Epithelioid Angiosarcoma in a Patient with Klippel-Trénaunay-Weber Syndrome: An Unexpected Response to Therapy
Identifieur interne : 002153 ( Pmc/Corpus ); précédent : 002152; suivant : 002154Epithelioid Angiosarcoma in a Patient with Klippel-Trénaunay-Weber Syndrome: An Unexpected Response to Therapy
Auteurs : Ângela Simas ; Catarina Matos ; Rodrigo Lopes Da Silva ; Vítor Brotas ; Eugénio Te Filo ; José Pereira AlbinoSource :
- Case Reports in Oncology [ 1662-6575 ] ; 2010.
Abstract
We present a rare case of Stewart-Treves syndrome characterized by a diffuse angiosarcoma of the leg in a 22-year-old man with a history of chronic lymphedema due to Klippel-Trénaunay-Weber syndrome. He underwent limb disarticulation and medical treatment with cycles of doxorubicin, oral thalidomide and sunitinib with a very good response after 12 months of follow-up.
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DOI: 10.1159/000313990
PubMed: 20740188
PubMed Central: 2919991
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Epithelioid Angiosarcoma in a Patient with Klippel-Trénaunay-Weber Syndrome: An Unexpected Response to Therapy</title><author><name sortKey="Simas, Angela" sort="Simas, Angela" uniqKey="Simas A" first="Ângela" last="Simas">Ângela Simas</name><affiliation><nlm:aff id="aff1">Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</nlm:aff></affiliation></author><author><name sortKey="Matos, Catarina" sort="Matos, Catarina" uniqKey="Matos C" first="Catarina" last="Matos">Catarina Matos</name><affiliation><nlm:aff id="aff3">Serviço de Medicina Interna 2, Hospital Pulido Valente, Lisboa, Portugal</nlm:aff></affiliation></author><author><name sortKey="Lopes Da Silva, Rodrigo" sort="Lopes Da Silva, Rodrigo" uniqKey="Lopes Da Silva R" first="Rodrigo" last="Lopes Da Silva">Rodrigo Lopes Da Silva</name><affiliation><nlm:aff id="aff1">Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</nlm:aff></affiliation><affiliation><nlm:aff id="aff2">Serviço de Hematologia Clínica, Hospital Santo António dos Capuchos</nlm:aff></affiliation></author><author><name sortKey="Brotas, Vitor" sort="Brotas, Vitor" uniqKey="Brotas V" first="Vítor" last="Brotas">Vítor Brotas</name><affiliation><nlm:aff id="aff1">Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</nlm:aff></affiliation></author><author><name sortKey="Te Filo, Eugenio" sort="Te Filo, Eugenio" uniqKey="Te Filo E" first="Eugénio" last="Te Filo">Eugénio Te Filo</name><affiliation><nlm:aff id="aff1">Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</nlm:aff></affiliation></author><author><name sortKey="Albino, Jose Pereira" sort="Albino, Jose Pereira" uniqKey="Albino J" first="José Pereira" last="Albino">José Pereira Albino</name><affiliation><nlm:aff id="aff4">Serviço de Cirurgia Vascular II, Hospital Pulido Valente, Lisboa, Portugal</nlm:aff></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">20740188</idno><idno type="pmc">2919991</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2919991</idno><idno type="RBID">PMC:2919991</idno><idno type="doi">10.1159/000313990</idno><date when="2010">2010</date><idno type="wicri:Area/Pmc/Corpus">002153</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002153</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Epithelioid Angiosarcoma in a Patient with Klippel-Trénaunay-Weber Syndrome: An Unexpected Response to Therapy</title><author><name sortKey="Simas, Angela" sort="Simas, Angela" uniqKey="Simas A" first="Ângela" last="Simas">Ângela Simas</name><affiliation><nlm:aff id="aff1">Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</nlm:aff></affiliation></author><author><name sortKey="Matos, Catarina" sort="Matos, Catarina" uniqKey="Matos C" first="Catarina" last="Matos">Catarina Matos</name><affiliation><nlm:aff id="aff3">Serviço de Medicina Interna 2, Hospital Pulido Valente, Lisboa, Portugal</nlm:aff></affiliation></author><author><name sortKey="Lopes Da Silva, Rodrigo" sort="Lopes Da Silva, Rodrigo" uniqKey="Lopes Da Silva R" first="Rodrigo" last="Lopes Da Silva">Rodrigo Lopes Da Silva</name><affiliation><nlm:aff id="aff1">Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</nlm:aff></affiliation><affiliation><nlm:aff id="aff2">Serviço de Hematologia Clínica, Hospital Santo António dos Capuchos</nlm:aff></affiliation></author><author><name sortKey="Brotas, Vitor" sort="Brotas, Vitor" uniqKey="Brotas V" first="Vítor" last="Brotas">Vítor Brotas</name><affiliation><nlm:aff id="aff1">Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</nlm:aff></affiliation></author><author><name sortKey="Te Filo, Eugenio" sort="Te Filo, Eugenio" uniqKey="Te Filo E" first="Eugénio" last="Te Filo">Eugénio Te Filo</name><affiliation><nlm:aff id="aff1">Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</nlm:aff></affiliation></author><author><name sortKey="Albino, Jose Pereira" sort="Albino, Jose Pereira" uniqKey="Albino J" first="José Pereira" last="Albino">José Pereira Albino</name><affiliation><nlm:aff id="aff4">Serviço de Cirurgia Vascular II, Hospital Pulido Valente, Lisboa, Portugal</nlm:aff></affiliation></author></analytic><series><title level="j">Case Reports in Oncology</title><idno type="eISSN">1662-6575</idno><imprint><date when="2010">2010</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>We present a rare case of Stewart-Treves syndrome characterized by a diffuse angiosarcoma of the leg in a 22-year-old man with a history of chronic lymphedema due to Klippel-Trénaunay-Weber syndrome. He underwent limb disarticulation and medical treatment with cycles of doxorubicin, oral thalidomide and sunitinib with a very good response after 12 months of follow-up.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Jacob, Ag" uniqKey="Jacob A">AG Jacob</name></author><author><name sortKey="Driscoll, Dj" uniqKey="Driscoll D">DJ Driscoll</name></author><author><name sortKey="Shaughnessy, Wj" uniqKey="Shaughnessy W">WJ Shaughnessy</name></author><author><name sortKey="Stanson, Aw" uniqKey="Stanson A">AW Stanson</name></author><author><name sortKey="Clay, Rp" uniqKey="Clay R">RP Clay</name></author><author><name sortKey="Gloviczki, P" uniqKey="Gloviczki P">P Gloviczki</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Berry, Sa" uniqKey="Berry S">SA Berry</name></author><author><name sortKey="Peterson, C" uniqKey="Peterson C">C Peterson</name></author><author><name sortKey="Mize, W" uniqKey="Mize 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<front><journal-meta><journal-id journal-id-type="nlm-ta">Case Rep Oncol</journal-id><journal-id journal-id-type="publisher-id">CRO</journal-id><journal-title-group><journal-title>Case Reports in Oncology</journal-title></journal-title-group><issn pub-type="epub">1662-6575</issn><publisher><publisher-name>S. Karger AG</publisher-name><publisher-loc>Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch</publisher-loc></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">20740188</article-id><article-id pub-id-type="pmc">2919991</article-id><article-id pub-id-type="doi">10.1159/000313990</article-id><article-id pub-id-type="publisher-id">cro0003-0148</article-id><article-categories><subj-group subj-group-type="heading"><subject>Published: April 2010</subject></subj-group></article-categories><title-group><article-title>Epithelioid Angiosarcoma in a Patient with Klippel-Trénaunay-Weber Syndrome: An Unexpected Response to Therapy</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>Simas</surname><given-names>Ângela</given-names></name><xref ref-type="aff" rid="aff1">a</xref></contrib><contrib contrib-type="author"><name><surname>Matos</surname><given-names>Catarina</given-names></name><xref ref-type="aff" rid="aff3">c</xref></contrib><contrib contrib-type="author"><name><surname>Lopes da Silva</surname><given-names>Rodrigo</given-names></name><xref ref-type="aff" rid="aff1">a</xref><xref ref-type="aff" rid="aff2">b</xref><xref ref-type="corresp" rid="cor1">*</xref></contrib><contrib contrib-type="author"><name><surname>Brotas</surname><given-names>Vítor</given-names></name><xref ref-type="aff" rid="aff1">a</xref></contrib><contrib contrib-type="author"><name><surname>Teófilo</surname><given-names>Eugénio</given-names></name><xref ref-type="aff" rid="aff1">a</xref></contrib><contrib contrib-type="author"><name><surname>Albino</surname><given-names>José Pereira</given-names></name><xref ref-type="aff" rid="aff4">d</xref></contrib></contrib-group><aff id="aff1"><sup>a</sup>Serviço de Medicina Interna 3, Hospital Santo António dos Capuchos</aff><aff id="aff2"><sup>b</sup>Serviço de Hematologia Clínica, Hospital Santo António dos Capuchos</aff><aff id="aff3"><sup>c</sup>Serviço de Medicina Interna 2, Hospital Pulido Valente, Lisboa, Portugal</aff><aff id="aff4"><sup>d</sup>Serviço de Cirurgia Vascular II, Hospital Pulido Valente, Lisboa, Portugal</aff><author-notes><corresp id="cor1">*Rodrigo Lopes da Silva, Serviço de Hematologia Clínica, Hospital Santo António dos Capuchos, Alameda Santo António dos Capuchos, PT–1169-050 Lisboa (Portugal), Tel. +351 21 313 6330, Fax +351 21 313 6490, E-Mail <email>ronolosi@gmail.com</email></corresp></author-notes><pub-date pub-type="collection"><season>Apr-Aug</season><year>2010</year></pub-date><pub-date pub-type="epub"><day>30</day><month>4</month><year>2010</year></pub-date><pub-date pub-type="pmc-release"><day>30</day><month>4</month><year>2010</year></pub-date><pmc-comment> PMC Release delay is 0 months and 0 days and was based on the
<volume>3</volume><issue>2</issue><fpage>148</fpage><lpage>153</lpage><permissions><copyright-statement>Copyright © 2010 by S. Karger AG, Basel</copyright-statement><copyright-year>2010</copyright-year><license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc-nd/3.0/"><license-p><pmc-comment>CREATIVE COMMONS</pmc-comment>This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License (<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by-nc-nd/3.0/">http://creativecommons.org/licenses/by-nc-nd/3.0/</ext-link>). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on <ext-link ext-link-type="uri" xlink:href="http://www.karger.com">http://www.karger.com</ext-link> and the terms of this license are included in any shared versions.</license-p></license></permissions><abstract><p>We present a rare case of Stewart-Treves syndrome characterized by a diffuse angiosarcoma of the leg in a 22-year-old man with a history of chronic lymphedema due to Klippel-Trénaunay-Weber syndrome. He underwent limb disarticulation and medical treatment with cycles of doxorubicin, oral thalidomide and sunitinib with a very good response after 12 months of follow-up.</p></abstract><kwd-group><title>Key Words</title><kwd>Angiosarcoma</kwd><kwd>Chemotherapy</kwd><kwd>Chronic lymphedema</kwd><kwd>Klippel-Trénaunay-Weber syndrome</kwd><kwd>Stewart-Treves syndrome</kwd></kwd-group><counts><fig-count count="4"></fig-count><ref-count count="15"></ref-count><page-count count="6"></page-count></counts></article-meta></front><body><sec sec-type="intro" id="sec1_1"><title>Introduction</title><p>Angiosarcoma that develops on a limb with chronic lymphedema is called Stewart-Treves syndrome. Usually, this appears as a complication of a long course lymphedema located on the arm, after mastectomy and/or radiotherapy due to breast cancer. Other reported types of chronic lymphedema in the Stewart-Treves syndrome include congenital lymphedema and lymphedema caused by venous thrombosis, trauma, infection (e.g. filariasis) and arteriovenous fistulae. In the latter case, venous hypertension, ulcers, infections, lymphagitis and angiosarcoma may develop. We present one case of this syndrome. It is a rare case, since it included a diffuse angiosarcoma of the leg in a 22-year-old man with a history of lymphedema due to Klippel-Trénaunay-Weber syndrome (KTWS). He underwent limb disarticulation and medical treatment with cycles of doxorubicin, oral thalidomide and sunitinib with a very good response after 12 months of follow-up.</p><p>Due to the aggressive nature of this syndrome, knowledge and research on its treatment are necessary.</p></sec><sec sec-type="cases" id="sec1_2"><title>Case Report</title><p>A 22-year-old black male from Guinea-Bissau was admitted complaining of progressive enlargement of the right lower limb since he was 14 years old. In the last two years, he had had recurring episodes of bleeding and pain and infected skin ulcers in that limb. There were no family or positive epidemiologic findings. The physical examination showed gigantic hemihypertrophy of his right lower limb, with multiple variegated and grape-like, red or purple, firm subcutaneous nodules in the calf and thigh, pinpoint red macules, some in a circle around the limb and multiple infected skin ulcers with excessive bleeding (<xref ref-type="fig" rid="F1">fig. 1</xref>). Laboratory tests revealed ferropenic anemia (Hb 6 g/dl; ferritin 3 ug/l). The search for microfilariae and filarial antibodies in the blood, serologies for HIV, HBV, and HCV, as well as VDRL and TPHA were negative. Serologic screening of HHV8 was positive. A culture of skin biopsy ruled out fungus and mycobacterium infections. A limb CT scan revealed marked thickening of the subcutaneous soft tissue, an increased intermuscular fat layer, lymphedema, extensive varicosities of the superficial and deep veins and massively dilated ileofemoral vessels (<xref ref-type="fig" rid="F2">fig. 2</xref>). A chest CT scan and ecochardiogram showed high output cardiopathy with severe pulmonary hyperemia. Upper and lower endoscopy showed multiple vascular ectases of the stomach, descending colon and rectal mucosae. Arteriography revealed multiple pelvic and lower-limb arteriovenous fistulae. The diagnosis of KTWS was made. Because of uncontrolled bleeding of the lower right limb, persistent ulcer infections and excessive pain, he was submitted to limb disarticulation. Surprisingly, the histopathologic examination of a nodule revealed an epithelioid angiosarcoma (<xref ref-type="fig" rid="F3">fig. 3</xref>), confirmed in other nodules, also from the contralateral leg. Repeated chest CT scan showed two new nodular lung images suspicious of being metastatic. Before starting treatment, we performed a PET scan that showed evidence of extensive disease with significant pelvic, left leg, upper abdomen and chest involvement (<xref ref-type="fig" rid="F4">fig. 4</xref>). Chemotherapy was started with oral thalidomide 200 mg/day, liposomal doxorubicin 30 mg (20 mg/m<sup>2</sup> body surface) every 21 days (5 cycles), and sunitinib 25 mg/day for 4 weeks followed by 2 weeks off treatment (4 cycles) was started. Laser therapy over new patches was given. The nodules have regressed. Physical rehabilitation was made with adaptation to a prosthetic right limb and a great improvement in quality of life. A second PET scan at 4 months’ follow-up showed active lesions just on the cicatricial area of the surgery.</p></sec><sec sec-type="discussion" id="sec1_3"><title>Discussion</title><p>KTWS is a rare syndrome with about 1,500 cases recorded [<xref ref-type="bibr" rid="B1">1</xref>] that manifests itself at birth or during childhood or adolescence. The lower limb is the site of malformations in approximately 95% of the patients [<xref ref-type="bibr" rid="B2">2</xref>]. The enlargement of the extremity consists of bone elongation and circumferential soft tissue hypertrophy. Vascular malformations involving the gastrointestinal and genitourinary tracts have been described [<xref ref-type="bibr" rid="B3">3</xref>]. Complications include stasis dermatitis, coagulopathy, pulmonary embolism, congestive heart failure and bleeding from abnormal vessels, including the gut, kidney and genitalia. However, the mortality of this syndrome is about 1% [<xref ref-type="bibr" rid="B1">1</xref>]. In a majority of patients, treatment is conservative (graded compressive stockings or pneumatic compression devices), but surgical treatment (epiphysiodesis, excision of soft tissue hypertrophy, percutaneous sclerosis or stripping of superficial varicose veins) may be indicated. In this patient, an extreme example of the natural course of KTWS, the only therapeutic option to control bleeding and painful ulcer infections was limb disarticulation.</p><p>Soft tissue sarcomas are very rare tumors (1% of adult malignant neoplasias) [<xref ref-type="bibr" rid="B4">4</xref>]. Most cases of angiosarcoma are associated with chronic lymphedema (Stewart-Treves syndrome, congenital lymphedema, filariasis) or with radiation exposure. Arteriovenous fistulae result in venous hypertension that may be complicated with lymphedema, ulcers, infection and lymphangitis. The lymphedematous region becomes an immunologically vulnerable area, predisposed to malignancy, because of the continual angiogenic stimulus [<xref ref-type="bibr" rid="B5">5</xref>]. The epithelioid angiosarcoma is a variant composed predominantly of large rounded ‘epithelioid’ endothelial cells with abundant amphophilic and eosinophilic cytoplasm and large vesicular nuclei [<xref ref-type="bibr" rid="B6">6</xref>]. Surgery in combination with radiotherapy is used for initial lesions. The chronic lymphedema-associated angiosarcoma has a very aggressive behaviour, with longer survival after radical amputation of the affected member [<xref ref-type="bibr" rid="B7">7</xref>]. Perfusion with tumor necrosis factor (TNF)-alpha and melphalan in the isolated limb has emerged as a new limb-salvage strategy [<xref ref-type="bibr" rid="B8">8</xref>]. Chemotherapy is indicated for disseminated tumours (doxorubicin, cyclophosphamide, methotrexate, vincristine) [<xref ref-type="bibr" rid="B9">9</xref>]. The prognosis is poor with a high recurrence rate, 84% at 5 years, and a high hematogenous metastasis rate [<xref ref-type="bibr" rid="B9">9</xref>]. One half of all patients may be expected to die within the first year after diagnosis with metastatic disease [<xref ref-type="bibr" rid="B6">6</xref>].</p><p>Our patient already had severe limb complications and disseminated disease. Owing to the rarity of the disease there are no treatment guidelines. We decided to start chemotherapy with doxorubicin (already approved for angiosarcoma) and thalidomide, which had already shown efficacy in four case reports [<xref ref-type="bibr" rid="B10">10</xref>,<xref ref-type="bibr" rid="B11">11</xref>,<xref ref-type="bibr" rid="B12">12</xref>,<xref ref-type="bibr" rid="B13">13</xref>]. The decision to add sunitinib, a novel tyrosine kinase inhibitor already approved for metastatic renal cell carcinoma and gastrointestinal stromal tumors, was based on its anti-tumor and antiangiogenic activities [<xref ref-type="bibr" rid="B14">14</xref>].</p></sec><sec sec-type="conclusion" id="sec1_4"><title>Conclusion</title><p>Our case illustrates an unexpected presentation of an epithelioid angiosarcoma, good therapeutic response with a 12-month survival and a great improvement in the quality of life. Interestingly, our patient gained 20 kg in 12 months and is actually on good health.</p><p>As far as we know, this is the first case of KTWS managed this way and with such good results. We wonder about the relationship between the HHV8 and angiosarcoma [<xref ref-type="bibr" rid="B15">15</xref>].</p></sec><sec id="sec1_5"><title>Authors’ Contribution</title><p>AS, CM, RL, VB, ET and JPA cared for the patient during his hospital admission, researched the topic, wrote/organized the paper and prepared the images. 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Gigantic hemihypertrophy of the right lower limb, with multiple variegated and grape-like, red or purple, firm subcutaneous nodules in the calf and thigh, pinpoint red macules, some in a circle around the limb and multiple infected skin ulcers with excessive bleeding.</p></caption><graphic xlink:href="cro0003-0148-f01"></graphic></fig><fig id="F2" orientation="portrait" position="float"><label>Fig. 2</label><caption><p>Limb CT scan. Marked thickening of the subcutaneous soft tissue, increased intermuscular fat layer, lymphedema, extensive varicosities of the superficial and deep veins and massively dilated ileofemoral vessels.</p></caption><graphic xlink:href="cro0003-0148-f02"></graphic></fig><fig id="F3" orientation="portrait" position="float"><label>Fig. 3</label><caption><p>Histopathologic examination of a lesion. On the left (HE, 400×): dilated vascular spaces lined by plump, atypical endothelial cells. On the right (Factor VIII, 400×): Immunohistochemical positive stain for Factor VIII.</p></caption><graphic xlink:href="cro0003-0148-f03"></graphic></fig><fig id="F4" orientation="portrait" position="float"><label>Fig. 4</label><caption><p>PET scan. Extensive disease with significant pelvic, left leg, upper abdomen and chest involvement.</p></caption><graphic xlink:href="cro0003-0148-f04"></graphic></fig></floats-group></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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