Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases
Identifieur interne : 000541 ( Pmc/Corpus ); précédent : 000540; suivant : 000542Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases
Auteurs : Ayodeji Oluwarotimi OmiyaleSource :
- Translational Andrology and Urology [ 2223-4691 ] ; 2015.
Abstract
Angiosarcoma (AS) is a malignant tumor occurring in less than 2% of soft tissue sarcomas. Primary involvement of the kidney is rare, its pathogenesis remains largely unknown and it has overlapping features with other tumors of the kidney. The objective of this paper is to review the case reports of primary AS of the kidney in the literature. The search terms were primary AS of the kidney, primary renal AS and primary renal hemangiosarcoma. The total cohort of the cases reviewed was 62. The mean age of presentation was 61 years old with a predilection for the male sex. Metastatic disease at the time of diagnosis accounted for 44.9% (22/49) of the cases reported and 44.4% (12/27) of patients with non-metastatic disease at diagnosis, subsequently developed metastasis. Primary AS of the kidney is a rare malignant tumor with a poor prognosis. Local recurrence and distant metastasis is common. Primary AS of the kidney shares similar clinical presentation with other renal tumors and imaging does not allow for tumor specific diagnosis. Histopathological examination and immunohistochemistry is very important for the confirmation of the diagnosis. Current treatment options include a variable combination of surgery, radiotherapy and chemotherapy.
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DOI: 10.3978/j.issn.2223-4683.2015.05.04
PubMed: 26816844
PubMed Central: 4708591
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases</title><author><name sortKey="Omiyale, Ayodeji Oluwarotimi" sort="Omiyale, Ayodeji Oluwarotimi" uniqKey="Omiyale A" first="Ayodeji Oluwarotimi" last="Omiyale">Ayodeji Oluwarotimi Omiyale</name></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">26816844</idno><idno type="pmc">4708591</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4708591</idno><idno type="RBID">PMC:4708591</idno><idno type="doi">10.3978/j.issn.2223-4683.2015.05.04</idno><date when="2015">2015</date><idno type="wicri:Area/Pmc/Corpus">000541</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000541</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases</title><author><name sortKey="Omiyale, Ayodeji Oluwarotimi" sort="Omiyale, Ayodeji Oluwarotimi" uniqKey="Omiyale A" first="Ayodeji Oluwarotimi" last="Omiyale">Ayodeji Oluwarotimi Omiyale</name></author></analytic><series><title level="j">Translational Andrology and Urology</title><idno type="eISSN">2223-4691</idno><imprint><date when="2015">2015</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Angiosarcoma (AS) is a malignant tumor occurring in less than 2% of soft tissue sarcomas. Primary involvement of the kidney is rare, its pathogenesis remains largely unknown and it has overlapping features with other tumors of the kidney. The objective of this paper is to review the case reports of primary AS of the kidney in the literature. The search terms were primary AS of the kidney, primary renal AS and primary renal hemangiosarcoma. The total cohort of the cases reviewed was 62. The mean age of presentation was 61 years old with a predilection for the male sex. Metastatic disease at the time of diagnosis accounted for 44.9% (22/49) of the cases reported and 44.4% (12/27) of patients with non-metastatic disease at diagnosis, subsequently developed metastasis. Primary AS of the kidney is a rare malignant tumor with a poor prognosis. Local recurrence and distant metastasis is common. Primary AS of the kidney shares similar clinical presentation with other renal tumors and imaging does not allow for tumor specific diagnosis. Histopathological examination and immunohistochemistry is very important for the confirmation of the diagnosis. Current treatment options include a variable combination of surgery, radiotherapy and chemotherapy.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Leggio, L" uniqKey="Leggio L">L Leggio</name></author><author><name sortKey="Addolorato, G" uniqKey="Addolorato G">G Addolorato</name></author><author><name sortKey="Abenavoli, L" uniqKey="Abenavoli L">L Abenavoli</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Brown, Jg" uniqKey="Brown J">JG Brown</name></author><author><name sortKey="Folpe, Al" uniqKey="Folpe A">AL Folpe</name></author><author><name sortKey="Rao, P" uniqKey="Rao P">P Rao</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Chaabouni, A" uniqKey="Chaabouni A">A Chaabouni</name></author><author><name sortKey="Rebai, N" uniqKey="Rebai N">N Rebai</name></author><author><name sortKey="Chabchoub, K" uniqKey="Chabchoub K">K Chabchoub</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Fukunaga, M" uniqKey="Fukunaga M">M. 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<front><journal-meta><journal-id journal-id-type="nlm-ta">Transl Androl Urol</journal-id><journal-id journal-id-type="iso-abbrev">Transl Androl Urol</journal-id><journal-id journal-id-type="publisher-id">TAU</journal-id><journal-title-group><journal-title>Translational Andrology and Urology</journal-title></journal-title-group><issn pub-type="epub">2223-4691</issn><publisher><publisher-name>AME Publishing Company</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">26816844</article-id><article-id pub-id-type="pmc">4708591</article-id><article-id pub-id-type="publisher-id">tau-04-04-464</article-id><article-id pub-id-type="doi">10.3978/j.issn.2223-4683.2015.05.04</article-id><article-categories><subj-group subj-group-type="heading"><subject>Review Article</subject></subj-group></article-categories><title-group><article-title>Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases</article-title></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name><surname>Omiyale</surname><given-names>Ayodeji Oluwarotimi</given-names></name></contrib><aff id="aff1">Department of Surgery, Heart of England NHS Trust, Birmingham Heartlands Hospital, Birmingham,<country>UK</country></aff></contrib-group><author-notes><corresp id="cor1"><italic>Correspondence to:</italic> Ayodeji Oluwarotimi Omiyale. Department of Surgery, Heart of England NHS Trust, Birmingham Heartlands Hospital, Birmingham, UK. Email: <email xlink:href="ayodejiomiyale@yahoo.com">ayodejiomiyale@yahoo.com</email>.</corresp></author-notes><pub-date pub-type="epub-ppub"><month>8</month><year>2015</year></pub-date><pmc-comment>Fake ppub date generated by PMC from publisher
pub-date/@pub-type='epub-ppub' </pmc-comment>
<pub-date pub-type="ppub"><month>8</month><year>2015</year></pub-date><volume>4</volume><issue>4</issue><fpage>464</fpage><lpage>473</lpage><history><date date-type="received"><day>17</day><month>2</month><year>2015</year></date><date date-type="accepted"><day>24</day><month>4</month><year>2015</year></date></history><permissions><copyright-statement>2015 Translational Andrology and Urology. All rights reserved.</copyright-statement><copyright-year>2015</copyright-year><copyright-holder>Translational Andrology and Urology.</copyright-holder></permissions><abstract><p>Angiosarcoma (AS) is a malignant tumor occurring in less than 2% of soft tissue sarcomas. Primary involvement of the kidney is rare, its pathogenesis remains largely unknown and it has overlapping features with other tumors of the kidney. The objective of this paper is to review the case reports of primary AS of the kidney in the literature. The search terms were primary AS of the kidney, primary renal AS and primary renal hemangiosarcoma. The total cohort of the cases reviewed was 62. The mean age of presentation was 61 years old with a predilection for the male sex. Metastatic disease at the time of diagnosis accounted for 44.9% (22/49) of the cases reported and 44.4% (12/27) of patients with non-metastatic disease at diagnosis, subsequently developed metastasis. Primary AS of the kidney is a rare malignant tumor with a poor prognosis. Local recurrence and distant metastasis is common. Primary AS of the kidney shares similar clinical presentation with other renal tumors and imaging does not allow for tumor specific diagnosis. Histopathological examination and immunohistochemistry is very important for the confirmation of the diagnosis. Current treatment options include a variable combination of surgery, radiotherapy and chemotherapy.</p></abstract><kwd-group kwd-group-type="author"><title>Keywords: </title><kwd>Angiosarcoma of the kidney</kwd><kwd>primary renal angiosarcoma (primary renal AS)</kwd><kwd>primary renal hemangiosarcoma</kwd></kwd-group></article-meta></front><body><sec sec-type="intro"><title>Introduction</title><p>Angiosarcoma (AS) is a rare and aggressive malignant tumor of vascular or lymphatic origin which accounts for <2% of soft tissue sarcomas. Approximately one third of AS occurs in the skin, one third in soft tissues and one third in other sites such as the liver, bone and breast (<xref rid="r1" ref-type="bibr">1</xref>-<xref rid="r4" ref-type="bibr">4</xref>). This paper reviews the literature for primary AS of the kidney because of its rarity and its overlapping features with other renal tumors which poses diagnostic challenges with implications for management.</p></sec><sec sec-type="methods"><title>Methods</title><p>PubMed, Scopus, Google Scholar and Embase (Ovid SP) databases were searched for all articles and case reports of primary AS of the kidney until April 2015. The search strategy combined medical subject heading (MeSH) descriptors and text words such as primary AS of the kidney, primary renal AS and renal hemangiosarcoma. There was no language limitation to the search and a manual search of the reference list of relevant articles was undertaken. Clinical and pathological data were extracted when available and analyzed. The clinical data included age, sex, clinical presentation, presence of metastasis, sites of metastasis, treatment modality and follow up. Pathological data included the maximum tumor dimension measured in centimeters, the laterality of the tumor and immunohistochemical (IHC) expression of the tumor cells. All cases reported prior to Prince <italic>et al.</italic> (<xref rid="r5" ref-type="bibr">5</xref>) in 1942 were excluded from this review because the definition of AS had not been clearly established at that time and this is consistent with the views of Cason <italic>et al.</italic> (<xref rid="r6" ref-type="bibr">6</xref>), Hiratsuka <italic>et al.</italic> (<xref rid="r7" ref-type="bibr">7</xref>) and Leggio <italic>et al.</italic> (<xref rid="r1" ref-type="bibr">1</xref>) in earlier reviews.</p></sec><sec sec-type="results"><title>Results</title><p><xref ref-type="table" rid="t1"><italic>Tables 1</italic></xref> and <xref ref-type="table" rid="t2"><italic>2</italic></xref> present a summary of the clinical and pathological data of the 62 cases of primary AS of the kidney in the literature.</p><table-wrap id="t1" orientation="portrait" position="float"><label>Table 1</label><caption><title>Clinical features of the reviewed cases of primary angiosarcoma of the kidney</title></caption><table frame="hsides" rules="groups"><col width="18.9%" span="1"></col><col width="9.39%" span="1"></col><col width="5.12%" span="1"></col><col width="5.12%" span="1"></col><col width="22.2%" span="1"></col><col width="8.54%" span="1"></col><col width="9.39%" span="1"></col><col width="21.34%" span="1"></col><thead><tr><th valign="middle" align="left" scope="col" rowspan="1" colspan="1">Author</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Age (years)</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Sex</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Side</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Clinical presentation</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Size (cm)</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Mets at dx</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Sites (initial and subsequent mets)</th></tr></thead><tbody><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Qayyum <italic>et al.</italic> (<xref rid="r8" ref-type="bibr">8</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">86</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Weight loss, fatigue, dizziness</td><td valign="middle" align="center" rowspan="1" colspan="1">12.3</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver, lungs</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Zhang <italic>et al.</italic> (<xref rid="r9" ref-type="bibr">9</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">52</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Left flank pain, left leg pain</td><td valign="middle" align="center" rowspan="1" colspan="1">8</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver, bone</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Liu <italic>et al.</italic> (<xref rid="r10" ref-type="bibr">10</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">75</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">4</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">68</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">24</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">64</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">30</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">71</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">10</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Peritoneal and periaortic LN</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">72</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">29</td><td valign="middle" align="center" rowspan="1" colspan="1">F</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">3.7</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">62</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">67</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">95</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">15.5</td><td valign="middle" align="center" rowspan="1" colspan="1">Na</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Cason <italic>et al.</italic> (<xref rid="r6" ref-type="bibr">6</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">46</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, weight loss</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Bone, liver, abdominal soft tissue</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Allred <italic>et al.</italic> (<xref rid="r11" ref-type="bibr">11</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">67</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Lungs, liver</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Peters <italic>et al.</italic> (<xref rid="r12" ref-type="bibr">12</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">74</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Weight loss</td><td valign="middle" align="center" rowspan="1" colspan="1">19</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Lungs, liver, abdominal wall, head</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Prince <italic>et al.</italic> (<xref rid="r5" ref-type="bibr">5</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">51</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">10</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Sabharwal <italic>et al.</italic> (<xref rid="r13" ref-type="bibr">13</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">67</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Loin pain, weight loss, loss of appetite</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Spleen</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Chaabouni <italic>et al.</italic> (<xref rid="r3" ref-type="bibr">3</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">59</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">6.5</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">López Cubillana <italic>et al.</italic> (<xref rid="r14" ref-type="bibr">14</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">72</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Bone and lung</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Singh <italic>et al.</italic> (<xref rid="r15" ref-type="bibr">15</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">83</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Askari <italic>et al.</italic> (<xref rid="r16" ref-type="bibr">16</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">24</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria, ureteric obstruction</td><td valign="middle" align="center" rowspan="1" colspan="1">9</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Douard <italic>et al.</italic> (<xref rid="r17" ref-type="bibr">17</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">60</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Bone, lung</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Terris <italic>et al.</italic> (<xref rid="r18" ref-type="bibr">18</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">47</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain</td><td valign="middle" align="center" rowspan="1" colspan="1">19</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Diaphragm, renal artery, liver and bone</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Zenico <italic>et al.</italic> (<xref rid="r19" ref-type="bibr">19</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">56</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Incidental finding</td><td valign="middle" align="center" rowspan="1" colspan="1">20</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Desai <italic>et al.</italic> (<xref rid="r20" ref-type="bibr">20</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">54</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, gingival bleed, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">21</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Bone</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Johnson <italic>et al.</italic> (<xref rid="r21" ref-type="bibr">21</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">50</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, hemoptysis</td><td valign="middle" align="center" rowspan="1" colspan="1">9</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver, lung</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Kern <italic>et al.</italic> (<xref rid="r22" ref-type="bibr">22</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">69</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, weight loss, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">26</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Lung</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Kern <italic>et al.</italic> (<xref rid="r22" ref-type="bibr">22</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">52</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">8</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Lung</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Fukunaga <italic>et al.</italic> (<xref rid="r4" ref-type="bibr">4</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">61</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Incidental</td><td valign="middle" align="center" rowspan="1" colspan="1">8</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver, bone and retroperitoneum</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Adjiman <italic>et al.</italic> (<xref rid="r23" ref-type="bibr">23</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">36</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Hemoptysis, flank pain, fever</td><td valign="middle" align="center" rowspan="1" colspan="1">10.5</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Chest wall, skin</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Papadimitriou<italic>et al.</italic> (<xref rid="r24" ref-type="bibr">24</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">68</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, dysuria, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">10</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Tsuda <italic>et al.</italic> (<xref rid="r25" ref-type="bibr">25</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">77</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria, renal failure</td><td valign="middle" align="center" rowspan="1" colspan="1">10</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Retroperitoneum, liver</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Leggio <italic>et al.</italic> (<xref rid="r1" ref-type="bibr">1</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">60</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Post-trauma abd pain</td><td valign="middle" align="center" rowspan="1" colspan="1">12</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Spleen, peritoneum</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Mordkin <italic>et al.</italic> (<xref rid="r26" ref-type="bibr">26</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">75</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Fever, weight loss</td><td valign="middle" align="center" rowspan="1" colspan="1">19</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Spleen, bone, liver, soft tissue, oral cavity</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Akkad <italic>et al.</italic> (<xref rid="r27" ref-type="bibr">27</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">58</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Incidental finding</td><td valign="middle" align="center" rowspan="1" colspan="1">4.5</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Cerilli <italic>et al.</italic> (<xref rid="r28" ref-type="bibr">28</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">67</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">12.5</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Renal vein</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Aksoy <italic>et al.</italic> (<xref rid="r29" ref-type="bibr">29</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">55</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Abd pain, rupture with retroperitoneal hematoma</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Hiratsuka <italic>et al.</italic> (<xref rid="r7" ref-type="bibr">7</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">59</td><td valign="middle" align="center" rowspan="1" colspan="1">F</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">4.5</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Aydogdu <italic>et al.</italic> (<xref rid="r30" ref-type="bibr">30</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">77</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Martínez-Piñeiro <italic>et al.</italic> (<xref rid="r31" ref-type="bibr">31</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">66</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Abd pain, anorexia, asthenia</td><td valign="middle" align="center" rowspan="1" colspan="1">11.5</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Bone, liver, lungs</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Lee <italic>et al.</italic> (<xref rid="r32" ref-type="bibr">32</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">63</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Berretta <italic>et al.</italic> (<xref rid="r33" ref-type="bibr">33</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">67</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Souza <italic>et al.</italic> (<xref rid="r34" ref-type="bibr">34</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">75</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Weight loss, flank pain, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">15</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Costero-Barrios <italic>et al.</italic> (<xref rid="r35" ref-type="bibr">35</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">71</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria, flank pain</td><td valign="middle" align="center" rowspan="1" colspan="1">18</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver and retroperitoneum</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Grapsa <italic>et al.</italic> (<xref rid="r36" ref-type="bibr">36</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">65</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Fever, fatigue, dyspnea</td><td valign="middle" align="center" rowspan="1" colspan="1">13.8</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver, lung</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Carnero López <italic>et al.</italic> (<xref rid="r37" ref-type="bibr">37</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">29</td><td valign="middle" align="center" rowspan="1" colspan="1">F</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Hemoptysis,</td><td valign="middle" align="center" rowspan="1" colspan="1">11</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Lungs</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Sesar <italic>et al.</italic> (<xref rid="r38" ref-type="bibr">38</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">65</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Incidental</td><td valign="middle" align="center" rowspan="1" colspan="1">4.5</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Pauli & Strutton (<xref rid="r39" ref-type="bibr">39</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">57</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Malaise, hemoptysis,</td><td valign="middle" align="center" rowspan="1" colspan="1">15</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Lungs, bone</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Yoshida <italic>et al.</italic> (<xref rid="r40" ref-type="bibr">40</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">78</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain</td><td valign="middle" align="center" rowspan="1" colspan="1">18</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver, bone</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Juan <italic>et al.</italic> (<xref rid="r41" ref-type="bibr">41</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">81</td><td valign="middle" align="center" rowspan="1" colspan="1">F</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">5</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Liver, bone</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Xuan (<xref rid="r42" ref-type="bibr">42</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">63</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Pain and costal swelling</td><td valign="middle" align="center" rowspan="1" colspan="1">10</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Yau <italic>et al.</italic> (<xref rid="r43" ref-type="bibr">43</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">38</td><td valign="middle" align="center" rowspan="1" colspan="1">F</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria, loin discomfort, bone pain</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Bone, LN,</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Garmendia <italic>et al.</italic> (<xref rid="r44" ref-type="bibr">44</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">51</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Nguyen <italic>et al.</italic> (<xref rid="r45" ref-type="bibr">45</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">53</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria, flank pain, malaise</td><td valign="middle" align="center" rowspan="1" colspan="1">7</td><td valign="middle" align="center" rowspan="1" colspan="1">Yes</td><td valign="middle" align="center" rowspan="1" colspan="1">Lung</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Limmer <italic>et al.</italic> (<xref rid="r46" ref-type="bibr">46</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">48</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain</td><td valign="middle" align="center" rowspan="1" colspan="1">10</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Lung, soft tissue, muscle</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Matter <italic>et al.</italic> (<xref rid="r47" ref-type="bibr">47</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">62</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, abd mass</td><td valign="middle" align="center" rowspan="1" colspan="1">18</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Lung</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Sanyal <italic>et al.</italic> (<xref rid="r48" ref-type="bibr">48</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">30</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Testa <italic>et al.</italic> (<xref rid="r49" ref-type="bibr">49</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Yamamoto <italic>et al.</italic> (<xref rid="r50" ref-type="bibr">50</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">68</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Incidental</td><td valign="middle" align="center" rowspan="1" colspan="1">7</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Rüb <italic>et al.</italic> (<xref rid="r51" ref-type="bibr">51</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">59</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria, weight loss</td><td valign="middle" align="center" rowspan="1" colspan="1">18</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Lungs, liver</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Celebi <italic>et al.</italic> (<xref rid="r52" ref-type="bibr">52</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">57</td><td valign="middle" align="center" rowspan="1" colspan="1">M</td><td valign="middle" align="center" rowspan="1" colspan="1">R</td><td valign="middle" align="center" rowspan="1" colspan="1">Flank pain, hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">14</td><td valign="middle" align="center" rowspan="1" colspan="1">No</td><td valign="middle" align="center" rowspan="1" colspan="1">Lung, pelvis</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Li <italic>et al.</italic> (<xref rid="r53" ref-type="bibr">53</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">69</td><td valign="middle" align="center" rowspan="1" colspan="1">F</td><td valign="middle" align="center" rowspan="1" colspan="1">L</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Witczak <italic>et al.</italic> (<xref rid="r54" ref-type="bibr">54</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">44</td><td valign="middle" align="center" rowspan="1" colspan="1">F</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">Hematuria</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr></tbody></table><table-wrap-foot><p>Mets, metastasis; dx, diagnosis; M, male; F, female; L, left; R, right; NA, not available; abd, abdominal; LN, lymph node.</p></table-wrap-foot></table-wrap><table-wrap id="t2" orientation="portrait" position="float"><label>Table 2</label><caption><title>Treatment and outcome of the reviewed cases of primary angiosarcoma of the kidney</title></caption><table frame="hsides" rules="groups"><col width="30.97%" span="1"></col><col width="30.82%" span="1"></col><col width="22.18%" span="1"></col><col width="16.03%" span="1"></col><thead><tr><th valign="middle" align="left" scope="col" rowspan="1" colspan="1">Author</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Treatment</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Follow-up (months)</th><th valign="middle" align="center" scope="col" rowspan="1" colspan="1">Outcome</th></tr></thead><tbody><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Qayyum <italic>et al.</italic> (<xref rid="r8" ref-type="bibr">8</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Palliative (patient’s wish)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Zhang <italic>et al.</italic> (<xref rid="r9" ref-type="bibr">9</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Liu <italic>et al.</italic> (<xref rid="r10" ref-type="bibr">10</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">6</td><td valign="middle" align="center" rowspan="1" colspan="1">NED</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">6</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">11</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">1</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">1</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Brown <italic>et al.</italic> (<xref rid="r2" ref-type="bibr">2</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">2</td><td valign="middle" align="center" rowspan="1" colspan="1">DFUD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Cason <italic>et al.</italic> (<xref rid="r6" ref-type="bibr">6</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">10</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Allred <italic>et al.</italic> (<xref rid="r11" ref-type="bibr">11</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">3</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Peters <italic>et al.</italic> (<xref rid="r12" ref-type="bibr">12</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">2</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Prince <italic>et al.</italic> (<xref rid="r5" ref-type="bibr">5</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">A&W</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Sabharwal <italic>et al.</italic> (<xref rid="r13" ref-type="bibr">13</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">1</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Chaabouni <italic>et al.</italic> (<xref rid="r3" ref-type="bibr">3</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">1</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">López Cubillana <italic>et al.</italic> (<xref rid="r14" ref-type="bibr">14</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">5</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Singh <italic>et al.</italic> (<xref rid="r15" ref-type="bibr">15</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Askari <italic>et al.</italic> (<xref rid="r16" ref-type="bibr">16</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">4</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Douard <italic>et al.</italic> (<xref rid="r17" ref-type="bibr">17</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">3</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Terris <italic>et al.</italic> (<xref rid="r18" ref-type="bibr">18</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">10</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Zenico <italic>et al.</italic> (<xref rid="r19" ref-type="bibr">19</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">4</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Desai <italic>et al.</italic> (<xref rid="r20" ref-type="bibr">20</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">4</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Johnson <italic>et al.</italic> (<xref rid="r21" ref-type="bibr">21</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Rapid deterioration</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Kern <italic>et al.</italic> (<xref rid="r22" ref-type="bibr">22</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">1.5</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Kern <italic>et al.</italic> (<xref rid="r22" ref-type="bibr">22</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">3</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Fukunaga <italic>et al.</italic> (<xref rid="r4" ref-type="bibr">4</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Adjiman <italic>et al.</italic> (<xref rid="r23" ref-type="bibr">23</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Papadimitriou <italic>et al.</italic> (<xref rid="r24" ref-type="bibr">24</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">A&W</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Tsuda <italic>et al.</italic> (<xref rid="r55" ref-type="bibr">55</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">21</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Leggio <italic>et al.</italic> (<xref rid="r1" ref-type="bibr">1</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">8</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Mordkin <italic>et al.</italic> (<xref rid="r26" ref-type="bibr">26</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy, S</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Akkad <italic>et al.</italic> (<xref rid="r27" ref-type="bibr">27</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">30</td><td valign="middle" align="center" rowspan="1" colspan="1">NED</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Cerilli <italic>et al.</italic> (<xref rid="r28" ref-type="bibr">28</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">6</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Aksoy <italic>et al.</italic> (<xref rid="r29" ref-type="bibr">29</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, S</td><td valign="middle" align="center" rowspan="1" colspan="1">3</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Hiratsuka <italic>et al.</italic> (<xref rid="r7" ref-type="bibr">7</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">29</td><td valign="middle" align="center" rowspan="1" colspan="1">NED</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Aydogdu <italic>et al.</italic> (<xref rid="r30" ref-type="bibr">30</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Martínez-Piñeiro <italic>et al.</italic> (<xref rid="r31" ref-type="bibr">31</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">5</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Lee <italic>et al.</italic> (<xref rid="r32" ref-type="bibr">32</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Berretta <italic>et al.</italic> (<xref rid="r33" ref-type="bibr">33</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Souza <italic>et al.</italic> (<xref rid="r34" ref-type="bibr">34</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">DFUD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Costero-Barrios <italic>et al.</italic> (<xref rid="r35" ref-type="bibr">35</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">12</td><td valign="middle" align="center" rowspan="1" colspan="1">Recurrence</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Grapsa <italic>et al.</italic> (<xref rid="r36" ref-type="bibr">36</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Carnero López <italic>et al.</italic> (<xref rid="r37" ref-type="bibr">37</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">5</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Sesar <italic>et al.</italic> (<xref rid="r38" ref-type="bibr">38</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephroureterectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Pauli & Strutton (<xref rid="r39" ref-type="bibr">39</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">2</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Yoshida <italic>et al.</italic> (<xref rid="r40" ref-type="bibr">40</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, immunotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Juan <italic>et al.</italic> (<xref rid="r41" ref-type="bibr">41</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">9</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Xuan (<xref rid="r42" ref-type="bibr">42</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Yau <italic>et al.</italic> (<xref rid="r43" ref-type="bibr">43</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">3</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Garmendia <italic>et al.</italic> (<xref rid="r44" ref-type="bibr">44</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Nguyen <italic>et al.</italic> (<xref rid="r45" ref-type="bibr">45</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">18</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Limmer <italic>et al.</italic> (<xref rid="r46" ref-type="bibr">46</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">1</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Matter <italic>et al.</italic> (<xref rid="r47" ref-type="bibr">47</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">18</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Sanyal <italic>et al.</italic> (<xref rid="r48" ref-type="bibr">48</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">24</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Testa <italic>et al.</italic> (<xref rid="r49" ref-type="bibr">49</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Yamamoto <italic>et al.</italic> (<xref rid="r50" ref-type="bibr">50</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, RT</td><td valign="middle" align="center" rowspan="1" colspan="1">19</td><td valign="middle" align="center" rowspan="1" colspan="1">NED</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Rüb <italic>et al.</italic> (<xref rid="r51" ref-type="bibr">51</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">12</td><td valign="middle" align="center" rowspan="1" colspan="1">AWD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Celebi <italic>et al.</italic> (<xref rid="r52" ref-type="bibr">52</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy, chemotherapy</td><td valign="middle" align="center" rowspan="1" colspan="1">13</td><td valign="middle" align="center" rowspan="1" colspan="1">DOD</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Li <italic>et al.</italic> (<xref rid="r53" ref-type="bibr">53</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr><tr><td valign="middle" align="left" scope="row" rowspan="1" colspan="1">Witczak <italic>et al.</italic> (<xref rid="r54" ref-type="bibr">54</xref>)</td><td valign="middle" align="center" rowspan="1" colspan="1">Nephrectomy</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td><td valign="middle" align="center" rowspan="1" colspan="1">NA</td></tr></tbody></table><table-wrap-foot><p>NED, no evidence of disease; NA, not available; DFUD, died from unrelated disease; AWD, alive with disease; DOD, died of disease; RT, radiotherapy; S, splenectomy; A&W, alive and well.</p></table-wrap-foot></table-wrap></sec><sec sec-type="discussion"><title>Discussion</title><p>Primary AS of the kidney is rare. Approximately 62 cases have been reported in the literature, mostly as case reports.</p><sec><title>Epidemiology</title><p>Primary AS of the kidney occurs most frequently in the sixth and seventh decades (<xref rid="r2" ref-type="bibr">2</xref>) and this is consistent with the findings of this review with a mean age of 61 years (range, 24-95 years). It has a predilection for the male sex which accounted for 89% (54/61) of the patients in the review. There are seven reports of female patients with primary AS of the kidney in the literature (<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r7" ref-type="bibr">7</xref>,<xref rid="r37" ref-type="bibr">37</xref>,<xref rid="r41" ref-type="bibr">41</xref>,<xref rid="r43" ref-type="bibr">43</xref>,<xref rid="r53" ref-type="bibr">53</xref>,<xref rid="r54" ref-type="bibr">54</xref>). The left kidney was involved in 66.7% (36/54) of the patients in this review. The laterality of the tumor was not documented for eight cases (<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r32" ref-type="bibr">32</xref>,<xref rid="r33" ref-type="bibr">33</xref>,<xref rid="r41" ref-type="bibr">41</xref>,<xref rid="r44" ref-type="bibr">44</xref>,<xref rid="r48" ref-type="bibr">48</xref>,<xref rid="r49" ref-type="bibr">49</xref>,<xref rid="r54" ref-type="bibr">54</xref>) and so far, no familial predisposition have been established in spite of the occurrence of the tumor in two brothers aged 52 and 69 years respectively (<xref rid="r22" ref-type="bibr">22</xref>).</p></sec><sec><title>Etiology</title><p>Although the etiology of primary AS of the kidney is not known, the association between some exogenous risk factors and AS of other sites, especially the liver is well documented. The risk factors include thorium dioxide (thorotrast used in the past for angiography), occupational exposure to arsenic in insecticide which is used in agriculture and polyvinyl chloride in synthetic rubber industry (<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r24" ref-type="bibr">24</xref>,<xref rid="r27" ref-type="bibr">27</xref>,<xref rid="r55" ref-type="bibr">55</xref>,<xref rid="r56" ref-type="bibr">56</xref>). Radiotherapy and chronic lymphedema of any cause, either due to Milroy’s disease or chronic infections like filariasis is also associated with the development of AS. A classical case in point is the phenomenon known as Stewart-Treves syndrome which describes AS associated with lymphedema that occurs after the treatment of breast cancer (<xref rid="r56" ref-type="bibr">56</xref>). However these risk factors have not been proven to have a direct causal relationship with AS of the kidney (<xref rid="r1" ref-type="bibr">1</xref>,<xref rid="r21" ref-type="bibr">21</xref>).</p></sec><sec><title>Clinical features</title><p>This review highlights the overlapping features between primary AS of the kidney and other renal tumors. Flank pain was the most common symptom (<xref rid="r3" ref-type="bibr">3</xref>,<xref rid="r5" ref-type="bibr">5</xref>,<xref rid="r6" ref-type="bibr">6</xref>,<xref rid="r9" ref-type="bibr">9</xref>,<xref rid="r11" ref-type="bibr">11</xref>,<xref rid="r18" ref-type="bibr">18</xref>,<xref rid="r20" ref-type="bibr">20</xref>-<xref rid="r24" ref-type="bibr">24</xref>,<xref rid="r28" ref-type="bibr">28</xref>,<xref rid="r29" ref-type="bibr">29</xref>,<xref rid="r31" ref-type="bibr">31</xref>,<xref rid="r34" ref-type="bibr">34</xref>,<xref rid="r35" ref-type="bibr">35</xref>,<xref rid="r40" ref-type="bibr">40</xref>,<xref rid="r46" ref-type="bibr">46</xref>,<xref rid="r47" ref-type="bibr">47</xref>,<xref rid="r52" ref-type="bibr">52</xref>). Hematuria with or without abdominal pain accounted for 45.6% (21/46) of the clinical presentation (<xref rid="r3" ref-type="bibr">3</xref>,<xref rid="r5" ref-type="bibr">5</xref>,<xref rid="r10" ref-type="bibr">10</xref>,<xref rid="r11" ref-type="bibr">11</xref>,<xref rid="r14" ref-type="bibr">14</xref>-<xref rid="r16" ref-type="bibr">16</xref>,<xref rid="r20" ref-type="bibr">20</xref>,<xref rid="r22" ref-type="bibr">22</xref>,<xref rid="r24" ref-type="bibr">24</xref>,<xref rid="r34" ref-type="bibr">34</xref>,<xref rid="r35" ref-type="bibr">35</xref>,<xref rid="r41" ref-type="bibr">41</xref>,<xref rid="r43" ref-type="bibr">43</xref>,<xref rid="r45" ref-type="bibr">45</xref>,<xref rid="r51" ref-type="bibr">51</xref>,<xref rid="r52" ref-type="bibr">52</xref>,<xref rid="r54" ref-type="bibr">54</xref>). Other clinical features included weight loss (<xref rid="r6" ref-type="bibr">6</xref>,<xref rid="r8" ref-type="bibr">8</xref>,<xref rid="r12" ref-type="bibr">12</xref>,<xref rid="r13" ref-type="bibr">13</xref>,<xref rid="r22" ref-type="bibr">22</xref>,<xref rid="r26" ref-type="bibr">26</xref>,<xref rid="r34" ref-type="bibr">34</xref>), fever (<xref rid="r23" ref-type="bibr">23</xref>,<xref rid="r26" ref-type="bibr">26</xref>,<xref rid="r36" ref-type="bibr">36</xref>), hemoptysis (<xref rid="r21" ref-type="bibr">21</xref>,<xref rid="r23" ref-type="bibr">23</xref>,<xref rid="r37" ref-type="bibr">37</xref>,<xref rid="r39" ref-type="bibr">39</xref>), flank or costal swelling (<xref rid="r42" ref-type="bibr">42</xref>), malaise and ureteric obstruction (<xref rid="r16" ref-type="bibr">16</xref>). One patient had spontaneous rupture with the development of retroperitoneal hematoma (<xref rid="r29" ref-type="bibr">29</xref>) and the tumor was an incidental finding in five patients (<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r19" ref-type="bibr">19</xref>,<xref rid="r27" ref-type="bibr">27</xref>,<xref rid="r38" ref-type="bibr">38</xref>,<xref rid="r50" ref-type="bibr">50</xref>).</p><p>Metastatic disease at the time of diagnosis accounted for 44.9% (22/49) of the cases reported and 44.4% (12/27) of the patients with non-metastatic disease at diagnosis subsequently developed metastasis. Approximately half of the patients with metastatic disease had two or more sites involved. The sites of tumor spread included the lung, liver, peritoneum, spleen, abdominal lymph nodes (LNs) and the soft tissues. The liver and the lungs were the most frequent site of metastasis and there were reports of metastatic disease of the bone (<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r6" ref-type="bibr">6</xref>,<xref rid="r9" ref-type="bibr">9</xref>,<xref rid="r14" ref-type="bibr">14</xref>,<xref rid="r17" ref-type="bibr">17</xref>,<xref rid="r18" ref-type="bibr">18</xref>,<xref rid="r20" ref-type="bibr">20</xref>,<xref rid="r26" ref-type="bibr">26</xref>,<xref rid="r31" ref-type="bibr">31</xref>,<xref rid="r39" ref-type="bibr">39</xref>-<xref rid="r41" ref-type="bibr">41</xref>,<xref rid="r43" ref-type="bibr">43</xref>).</p></sec><sec><title>Imaging</title><p>Radiological imaging alone cannot ascertain the diagnosis of AS of the kidney. It may appear as a large necrotic renal mass on computed tomography (CT) which is virtually indistinguishable from a renal cell carcinoma (<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r15" ref-type="bibr">15</xref>). Contrast-enhanced CT findings (<xref ref-type="fig" rid="f1"><italic>Figure 1</italic></xref>) have been variably described as heterogeneous mass with peripheral enhancement (<xref rid="r1" ref-type="bibr">1</xref>,<xref rid="r7" ref-type="bibr">7</xref>,<xref rid="r10" ref-type="bibr">10</xref>,<xref rid="r27" ref-type="bibr">27</xref>) or hypo-dense renal mass with areas of enhancement (<xref rid="r13" ref-type="bibr">13</xref>,<xref rid="r34" ref-type="bibr">34</xref>). CT imaging also helps in delineating metastatic deposits. Given the rarity of AS of the kidney and in the event of small multiple lesions to distant sites such as the lungs and liver, a large single renal mass will be suggestive of a primary AS of the kidney (<xref rid="r2" ref-type="bibr">2</xref>).</p><fig id="f1" fig-type="figure" orientation="portrait" position="float"><label>Figure 1</label><caption><p>CT imaging of primary angiosarcoma of the right kidney reprinted from (<xref rid="r8" ref-type="bibr">8</xref>) with the permission of the Editor-in-Chief of <italic>Case Reports in Pathology</italic>. CT, computed tomography.</p></caption><graphic xlink:href="tau-04-04-464-f1"></graphic></fig></sec><sec><title>Preoperative diagnosis</title><p>Three patients with primary AS of the kidney have been diagnosed with CT guided fine needle aspiration cytology and the fine needle aspiration (FNA) results correlated with the histopathological and immunohistochemistry findings (<xref rid="r15" ref-type="bibr">15</xref>,<xref rid="r21" ref-type="bibr">21</xref>,<xref rid="r36" ref-type="bibr">36</xref>).</p></sec><sec><title>Pathologic findings</title><p>The histopathological feature of primary AS of the kidney is similar to AS of other sites. The average size of the tumor in this review is 13 cm (range, 3.7-30 cm). Primary AS of the kidney is predominantly solitary lesions. A rare instance of multifocal lesions (three) of the left kidney which measured 0.5-4.5 cm in size has been reported (<xref rid="r38" ref-type="bibr">38</xref>). AS of the kidney is mostly a hemorrhagic (<xref rid="r1" ref-type="bibr">1</xref>,<xref rid="r3" ref-type="bibr">3</xref>,<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r9" ref-type="bibr">9</xref>,<xref rid="r10" ref-type="bibr">10</xref>,<xref rid="r13" ref-type="bibr">13</xref>,<xref rid="r19" ref-type="bibr">19</xref>,<xref rid="r25" ref-type="bibr">25</xref>,<xref rid="r35" ref-type="bibr">35</xref>) ill-defined (<xref rid="r7" ref-type="bibr">7</xref>,<xref rid="r10" ref-type="bibr">10</xref>) or well circumscribed necrotic renal mass (<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r8" ref-type="bibr">8</xref>) (<xref ref-type="fig" rid="f2"><italic>Figure 2</italic></xref>). The renal parenchyma may be destroyed with frequent tumor extension to the perinephric fatty tissue (<xref rid="r3" ref-type="bibr">3</xref>,<xref rid="r4" ref-type="bibr">4</xref>).</p><fig id="f2" fig-type="figure" orientation="portrait" position="float"><label>Figure 2</label><caption><p>Microscopic features demonstrating necrosis (H&E ×100) reprinted from (<xref rid="r8" ref-type="bibr">8</xref>) with the permission of the Editor-in-Chief of <italic>Case Reports in Pathology</italic>.</p></caption><graphic xlink:href="tau-04-04-464-f2"></graphic></fig><p>Primary AS of the kidney showed multiple and irregular anastomosing vascular spaces or channels which are lined by discrete and large endothelial cells with variable degrees of cytological pleomorphism, nuclear atypia, mitotic activity (<xref ref-type="fig" rid="f3"><italic>Figure 3</italic></xref>) and multilayering (<xref rid="r1" ref-type="bibr">1</xref>,<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r7" ref-type="bibr">7</xref>,<xref rid="r8" ref-type="bibr">8</xref>,<xref rid="r10" ref-type="bibr">10</xref>,<xref rid="r24" ref-type="bibr">24</xref>,<xref rid="r32" ref-type="bibr">32</xref>,<xref rid="r34" ref-type="bibr">34</xref>). There was a mix of epithelioid (<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r10" ref-type="bibr">10</xref>,<xref rid="r15" ref-type="bibr">15</xref>) and spindle cell (<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r10" ref-type="bibr">10</xref>,<xref rid="r15" ref-type="bibr">15</xref>,<xref rid="r28" ref-type="bibr">28</xref>,<xref rid="r40" ref-type="bibr">40</xref>) morphological pattern.</p><fig id="f3" fig-type="figure" orientation="portrait" position="float"><label>Figure 3</label><caption><p>Microscopic section of the tumour showing vascular channels lined with endothelial cells with varying degree of pleomorphism and mitotic activity (H&E ×400) reprinted from (<xref rid="r8" ref-type="bibr">8</xref>) with the permission of the Editor-in-Chief of <italic>Case Reports in Pathology</italic>.</p></caption><graphic xlink:href="tau-04-04-464-f3"></graphic></fig></sec><sec><title>IHC studies</title><p>Primary AS of the kidney reacts negatively for epithelial markers like epithelial membrane antigen (EMA), Cam 5.2 and AE1/AE3 (<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r8" ref-type="bibr">8</xref>). Although AS of the kidney is mostly negative for epithelial markers, positive expression have been infrequently encountered in AS of other sites. A study of 80 cases of AS of the soft tissue demonstrated immunoreactivity to cytokeratins in 35% of the cases (<xref rid="r57" ref-type="bibr">57</xref>). This observation may reflect the rarity of primary AS of the kidney and the paucity of comprehensive IHC staining. Some positivity to epithelial markers in AS of the kidney, similar to the soft tissue counterparts may be found if larger numbers of AS of the kidney were studied. This highlights the need for a cocktail of panel of antibodies or markers in the diagnostic workup rather than a single marker. The tumor stained negative for RCC, CK8/18 (<xref rid="r8" ref-type="bibr">8</xref>), CD10 (<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r8" ref-type="bibr">8</xref>,<xref rid="r27" ref-type="bibr">27</xref>), S100, Melan-A and HMB-45 (<xref rid="r1" ref-type="bibr">1</xref>,<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r9" ref-type="bibr">9</xref>,<xref rid="r28" ref-type="bibr">28</xref>). Most tumor cells stained positively for endothelial markers (<xref ref-type="fig" rid="f4"><italic>Figure 4</italic></xref>) such as CD31, CD34 (<xref rid="r1" ref-type="bibr">1</xref>,<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r8" ref-type="bibr">8</xref>,<xref rid="r9" ref-type="bibr">9</xref>,<xref rid="r15" ref-type="bibr">15</xref>,<xref rid="r24" ref-type="bibr">24</xref>,<xref rid="r27" ref-type="bibr">27</xref>,<xref rid="r32" ref-type="bibr">32</xref>,<xref rid="r34" ref-type="bibr">34</xref>,<xref rid="r40" ref-type="bibr">40</xref>), FLI-1 (<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r15" ref-type="bibr">15</xref>) and factor 8-related antigen (<xref rid="r3" ref-type="bibr">3</xref>,<xref rid="r4" ref-type="bibr">4</xref>,<xref rid="r7" ref-type="bibr">7</xref>,<xref rid="r14" ref-type="bibr">14</xref>,<xref rid="r15" ref-type="bibr">15</xref>,<xref rid="r27" ref-type="bibr">27</xref>,<xref rid="r28" ref-type="bibr">28</xref>,<xref rid="r34" ref-type="bibr">34</xref>,<xref rid="r40" ref-type="bibr">40</xref>). Co-expression of vimentin may be present (<xref rid="r3" ref-type="bibr">3</xref>,<xref rid="r10" ref-type="bibr">10</xref>,<xref rid="r19" ref-type="bibr">19</xref>,<xref rid="r40" ref-type="bibr">40</xref>).</p><fig id="f4" fig-type="figure" orientation="portrait" position="float"><label>Figure 4</label><caption><p>Immunohistochemical study of the tumour showing strong immunoreactivity for CD34 (IHC ×400) reprinted from (<xref rid="r8" ref-type="bibr">8</xref>) with the permission of the Editor-in-Chief of <italic>Case Reports in Pathology</italic>. IHC, immunohistochemical.</p></caption><graphic xlink:href="tau-04-04-464-f4"></graphic></fig><p>Aberrant expression of neuroendocrine markers in AS, which could be a potential diagnostic pitfall was recently documented (<xref rid="r58" ref-type="bibr">58</xref>). A case in point was the co-expression of synaptophysin in 5-10% of cells and chromogranin A in >75% of cells in a 29-year-old Afro-American lady with a hemorrhagic 3.7 cm AS of the kidney (<xref rid="r2" ref-type="bibr">2</xref>,<xref rid="r58" ref-type="bibr">58</xref>). Synaptophysin was positive in >75% of cells in the other two cases of AS reported. This finding suggested the possibility that some AS may show true neuroendocrine differentiation or it was simply a case of anomalous expression of the antigen. The aggressive clinical behavior of AS with aberrant neuroendocrine expression was similar to other AS however it remains unclear if the finding is of any clinical importance (<xref rid="r58" ref-type="bibr">58</xref>). The Ki-67 index of 30% (<xref rid="r10" ref-type="bibr">10</xref>), 40% (<xref rid="r9" ref-type="bibr">9</xref>) and >80% (<xref rid="r13" ref-type="bibr">13</xref>) is suggestive of the highly proliferative nature of the tumor.</p><p>Erythroblast transformation specific related gene (<italic>ERG</italic>), an ETS family transcription factor is an IHC marker with high sensitivity and specificity for vascular endothelial tumors. A study of 1,880 tumors which included vascular endothelial, mesenchymal and epithelial tumors that examined the diagnostic utility of <italic>ERG</italic>, suggested that <italic>ERG</italic> compares favorably with CD31 as a marker for AS. Ninety-six percent (96/100) AS of different clinicopathological subgroups and sites confirmed as CD31 positive, demonstrated nuclear <italic>ERG</italic> expression (<xref rid="r59" ref-type="bibr">59</xref>).</p><p>None of the cases in this review documented IHC study and expression of <italic>PAX8</italic> and <italic>PAX2</italic> markers. <italic>PAX8</italic> and <italic>PAX2</italic> belong to the family of paired box gene which encodes for nuclear transcription factors which is important in organogenesis and are excellent markers for tumors of renal, thyroid and mullerian origin (<xref rid="r60" ref-type="bibr">60</xref>). The expression of both markers have been documented in primary and metastatic renal epithelial tumors such as chromophobe RCC, oncocytomas, clear cell RCC, renal medullary carcinomas, papillary RCC etc. <italic>PAX8</italic> appears to be a more sensitive marker compared to <italic>PAX2</italic> (<xref rid="r61" ref-type="bibr">61</xref>). To the best of the knowledge of the author, the diagnostic utility of <italic>PAX8</italic> and <italic>PAX2</italic> in primary AS of the kidney awaits investigation.</p></sec><sec><title>Treatment</title><p>The rarity of primary AS of the kidney is largely responsible for the lack of standardized therapy. The patients were mostly treated with nephrectomy with varying combinations with chemotherapy (<xref rid="r6" ref-type="bibr">6</xref>,<xref rid="r11" ref-type="bibr">11</xref>,<xref rid="r13" ref-type="bibr">13</xref>,<xref rid="r14" ref-type="bibr">14</xref>,<xref rid="r20" ref-type="bibr">20</xref>,<xref rid="r26" ref-type="bibr">26</xref>,<xref rid="r33" ref-type="bibr">33</xref>,<xref rid="r35" ref-type="bibr">35</xref>,<xref rid="r37" ref-type="bibr">37</xref>,<xref rid="r41" ref-type="bibr">41</xref>,<xref rid="r43" ref-type="bibr">43</xref>,<xref rid="r45" ref-type="bibr">45</xref>,<xref rid="r47" ref-type="bibr">47</xref>,<xref rid="r51" ref-type="bibr">51</xref>,<xref rid="r52" ref-type="bibr">52</xref>), radiotherapy (<xref rid="r5" ref-type="bibr">5</xref>,<xref rid="r6" ref-type="bibr">6</xref>,<xref rid="r10" ref-type="bibr">10</xref>,<xref rid="r18" ref-type="bibr">18</xref>,<xref rid="r28" ref-type="bibr">28</xref>,<xref rid="r35" ref-type="bibr">35</xref>,<xref rid="r39" ref-type="bibr">39</xref>,<xref rid="r41" ref-type="bibr">41</xref>,<xref rid="r43" ref-type="bibr">43</xref>,<xref rid="r47" ref-type="bibr">47</xref>,<xref rid="r48" ref-type="bibr">48</xref>,<xref rid="r50" ref-type="bibr">50</xref>) and recombinant interleukin-2 therapy (<xref rid="r40" ref-type="bibr">40</xref>). The best treatment option for AS of the kidney remains controversial. Surgery appears to be the most effective treatment approach (<xref rid="r31" ref-type="bibr">31</xref>). Terris <italic>et al.</italic> (<xref rid="r18" ref-type="bibr">18</xref>) suggested that post-operative adjuvant radiotherapy may contribute to local control in a manner similar to AS of other sites, however Martínez-Piñeiro <italic>et al.</italic> (<xref rid="r31" ref-type="bibr">31</xref>) held a contrary view and observed that radiotherapy does not seem to prolong survival and chemotherapy should be added to the treatment. Mordkin <italic>et al.</italic> (<xref rid="r26" ref-type="bibr">26</xref>) suggested that chemotherapy may be used for palliative treatment, although the response is likely to be short. Zenico <italic>et al.</italic> (<xref rid="r19" ref-type="bibr">19</xref>) observed that patients who had the best response to treatment also underwent radiotherapy and chemotherapy with a median survival of 13 months (P>0.05) compared to 7 months in patients who underwent nephrectomy only. Zenico <italic>et al.</italic> however emphasized that none of the patients in the cohort reviewed who was treated with chemotherapy or radiotherapy had distant metastasis at the time of diagnosis (<xref rid="r19" ref-type="bibr">19</xref>).</p></sec><sec><title>Prognosis</title><p>Primary AS of the kidney is a very aggressive tumor with a poor prognosis. The mean follow-up is 7 months (range, 1-30 months) and most patients died of the disease. Data on the treatment outcome was not available for 18 of the patients in the review and that includes the case of a male patient who developed coincidental acute myeloblastic leukemia 7 months post left radical nephrectomy (<xref rid="r30" ref-type="bibr">30</xref>). There is evidence to suggest that the size of the tumor seems to be the most important factor for determining the prognosis of AS of the kidney. Tumors <5 cm have a significantly better prognosis compared to larger tumor lesions. Mark <italic>et al.</italic> (<xref rid="r62" ref-type="bibr">62</xref>) in a review of 67 cases of AS reported a 5-year survival of 32% for lesions <5 cm compared to 13% for lesions >5 cm. Consistent with this finding by Mark <italic>et al.</italic> are the two cases of primary AS of the kidney with the longest post-operative survival in the literature. Akkad <italic>et al.</italic> reported a patient with AS of the kidney 4.5 cm in diameter who had no evidence of disease 30 months after surgery without adjuvant therapy (<xref rid="r27" ref-type="bibr">27</xref>). Hiratsuka <italic>et al.</italic> also reported a patient with tumor size of 4.5 cm as well and the patient was disease free after 29 months of follow-up (<xref rid="r7" ref-type="bibr">7</xref>).</p></sec></sec><sec sec-type="conclusions"><title>Conclusions</title><p>Primary AS of the kidney is a rare malignant tumor with a poor prognosis. It has a propensity for both local recurrence and distant metastasis at the time of diagnosis or shortly afterwards. The pathogenesis remains unclear, it has overlapping features with other renal tumors and imaging does not allow for tumor specific diagnosis. The importance of histopathology and immunohistochemistry cannot be overemphasized in the diagnosis of the tumor. There is no optimal and standard treatment however, current treatment options include a variable combination of surgery, radiotherapy and chemotherapy.</p></sec></body><back><ack><title>Acknowledgements</title><p>Many thanks to the Editor-in-Chief of <italic>Case Reports in Pathology</italic> for the kind permission to use the images and figures reprinted from <italic>Primary renal angiosarcoma with extensive necrosis: a difficult diagnosis</italic>.</p></ack><fn-group><fn fn-type="conflict"><p><italic>Conflicts of Interest:</italic> The author has no conflicts of interest to declare.</p></fn></fn-group><ref-list><title>References</title><ref id="r1"><label>1</label><mixed-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Leggio</surname><given-names>L</given-names></name><name><surname>Addolorato</surname><given-names>G</given-names></name><name><surname>Abenavoli</surname><given-names>L</given-names></name><etal></etal></person-group><article-title>Primary renal angiosarcoma: a rare malignancy. 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