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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Desmoplastic Small Round Cell Tumor: Report of 2 Cases Treated with Chemotherapy Alone or in Combination with Bevacizumab</title><author><name sortKey="De Araujo, Rogerio Agenor" sort="De Araujo, Rogerio Agenor" uniqKey="De Araujo R" first="Rogério Agenor" last="De Araújo">Rogério Agenor De Araújo</name><affiliation><nlm:aff id="aff1">Department of Oncology, Federal University of Uberlândia, Uberlândia, Brazil</nlm:aff></affiliation></author><author><name sortKey="Araujo, Breno Jeha" sort="Araujo, Breno Jeha" uniqKey="Araujo B" first="Breno Jeha" last="Araújo">Breno Jeha Araújo</name><affiliation><nlm:aff>NONE</nlm:aff></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">24707256</idno><idno type="pmc">3975756</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3975756</idno><idno type="RBID">PMC:3975756</idno><idno type="doi">10.1159/000359997</idno><date when="2014">2014</date><idno type="wicri:Area/Pmc/Corpus">002146</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">002146</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Desmoplastic Small Round Cell Tumor: Report of 2 Cases Treated with Chemotherapy Alone or in Combination with Bevacizumab</title><author><name sortKey="De Araujo, Rogerio Agenor" sort="De Araujo, Rogerio Agenor" uniqKey="De Araujo R" first="Rogério Agenor" last="De Araújo">Rogério Agenor De Araújo</name><affiliation><nlm:aff id="aff1">Department of Oncology, Federal University of Uberlândia, Uberlândia, Brazil</nlm:aff></affiliation></author><author><name sortKey="Araujo, Breno Jeha" sort="Araujo, Breno Jeha" uniqKey="Araujo B" first="Breno Jeha" last="Araújo">Breno Jeha Araújo</name><affiliation><nlm:aff>NONE</nlm:aff></affiliation></author></analytic><series><title level="j">Case Reports in Oncology</title><idno type="eISSN">1662-6575</idno><imprint><date when="2014">2014</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Here, we present 2 case reports of patients with desmoplastic small round cell tumor (DSRCT), a very rare and aggressive mesenchymal cancer, and we discuss 2therapeutic options for this sarcoma. This report focuses on men aged 22 and 37 years, respectively. The first patient presented with an abdominopelvic mass which was not suitable for surgery. He underwent chemotherapy (adriblastina and cisplatin) with a brief partial remission and survival time of 13 months. The second patient presented with an abdominal mass and underwent partial resection. He received chemotherapy and bevacizumab, resulting in a partial remission and a survival time of 34 months. The extent of surgery and monoclonal antibody use probably had a positive impact on survival. It is necessary to include specific targeted therapies in an attempt to improve survival. Surprisingly, positron emission tomography was not effective in restaging of the second patient, emphasizing the importance of computed tomography or magnetic resonance in DSRCT.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Koniari, K" uniqKey="Koniari K">K Koniari</name></author><author><name sortKey="Mahera, H" uniqKey="Mahera H">H Mahera</name></author><author><name sortKey="Nikolaou, M" uniqKey="Nikolaou M">M Nikolaou</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Dufresne, A" uniqKey="Dufresne A">A Dufresne</name></author><author><name sortKey="Cassier, P" uniqKey="Cassier P">P Cassier</name></author><author><name sortKey="Couraud, L" uniqKey="Couraud L">L Couraud</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Perna, Mj" uniqKey="Perna M">MJ Perna</name></author><author><name sortKey="Streck, Cj" uniqKey="Streck C">CJ Streck</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Gerald, Wl" uniqKey="Gerald W">WL Gerald</name></author><author><name sortKey="Rosai, J" uniqKey="Rosai J">J Rosai</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey=" Rdo Ez, Ng" uniqKey=" Rdo Ez N">NG Órdoñez</name></author><author><name sortKey="El Naggar, Ak" uniqKey="El Naggar A">AK El-Naggar</name></author><author><name sortKey="Ro, Jy" uniqKey="Ro J">JY Ro</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Kretschmar, Cs" uniqKey="Kretschmar C">CS Kretschmar</name></author><author><name sortKey="Colbach, C" uniqKey="Colbach C">C Colbach</name></author><author><name sortKey="Bhan, I" uniqKey="Bhan I">I Bhan</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Gerald, Wl" uniqKey="Gerald W">WL Gerald</name></author><author><name sortKey="Ladanyi, M" uniqKey="Ladanyi M">M Ladanyi</name></author><author><name sortKey="Alava, E" uniqKey="Alava E">E Alava</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Ogata, Dc" uniqKey="Ogata D">DC Ogata</name></author><author><name sortKey="Tatsugui, Jt" uniqKey="Tatsugui J">JT Tatsugui</name></author><author><name sortKey="Machuca, Tn" uniqKey="Machuca T">TN Machuca</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Cao, L" uniqKey="Cao L">L Cao</name></author><author><name sortKey="Ni, J" uniqKey="Ni J">J Ni</name></author><author><name sortKey="Que, R" uniqKey="Que R">R Que</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Philippe Chomette, P" uniqKey="Philippe Chomette P">P Philippe-Chomette</name></author><author><name sortKey="Kabbara, N" uniqKey="Kabbara N">N Kabbara</name></author><author><name sortKey="Andre, N" uniqKey="Andre N">N Andre</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Torres, Us" uniqKey="Torres U">US Torres</name></author><author><name sortKey="Ribeiro, Mca" uniqKey="Ribeiro M">MCA Ribeiro</name></author><author><name sortKey="Souza, As" uniqKey="Souza A">AS Souza</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Kushner, Bh" uniqKey="Kushner B">BH Kushner</name></author><author><name sortKey="Laquaglia, Mp" uniqKey="Laquaglia M">MP LaQuaglia</name></author><author><name sortKey="Wollner, N" uniqKey="Wollner N">N Wollner</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Gil, A" uniqKey="Gil A">A Gil</name></author><author><name sortKey="Portilla, Ag" uniqKey="Portilla A">AG Portilla</name></author><author><name sortKey="Brun, Ea" uniqKey="Brun E">EA Brun</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Mrabti, H" uniqKey="Mrabti H">H Mrabti</name></author><author><name sortKey="Kaikani, W" uniqKey="Kaikani W">W Kaikani</name></author><author><name sortKey="Ahbeddou, N" uniqKey="Ahbeddou N">N Ahbeddou</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Westphalen, Aca" uniqKey="Westphalen A">ACA Westphalen</name></author><author><name sortKey="Ferreira, Jhp" uniqKey="Ferreira J">JHP Ferreira</name></author><author><name sortKey="Daudt, Aw" uniqKey="Daudt A">AW Daudt</name></author></analytic></biblStruct></listBibl></div1></back></TEI><pmc article-type="case-report"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">Case Rep Oncol</journal-id><journal-id journal-id-type="iso-abbrev">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">24707256</article-id><article-id pub-id-type="pmc">3975756</article-id><article-id pub-id-type="doi">10.1159/000359997</article-id><article-id pub-id-type="publisher-id">cro-0007-0102</article-id><article-categories><subj-group subj-group-type="heading"><subject>Published online: February, 2014</subject></subj-group></article-categories><title-group><article-title>Desmoplastic Small Round Cell Tumor: Report of 2 Cases Treated with Chemotherapy Alone or in Combination with Bevacizumab</article-title></title-group><contrib-group><contrib contrib-type="author"><name><surname>de Araújo</surname><given-names>Rogério Agenor</given-names></name><xref ref-type="aff" rid="aff1"><sup>a</sup></xref><xref ref-type="corresp" rid="cor1">*</xref></contrib><contrib contrib-type="author"><name><surname>Araújo</surname><given-names>Breno Jeha</given-names></name><xref ref-type="aff" rid="aff2"><sup>b</sup></xref></contrib></contrib-group><aff id="aff1"><sup>a</sup>Department of Oncology, Federal University of Uberlândia, Uberlândia, Brazil</aff><aff id="aff2"><sup>2</sup>School of Medicine, Federal University of Uberlândia, Uberlândia, Brazil</aff><author-notes><corresp id="cor1">*Rogério Agenor de Araújo, Department of Oncology, Universidade Federal de Uberlândia, Campus Umuarama, Bloco 4A, Uberlândia, MG 38400-000 (Brazil), E-Mail <email>rogeriodearaujo@gmail.com</email></corresp></author-notes><pub-date pub-type="collection"><season>Jan-Apr</season><year>2014</year></pub-date><pub-date pub-type="epub"><day>15</day><month>2</month><year>2014</year></pub-date><pub-date pub-type="pmc-release"><day>15</day><month>2</month><year>2014</year></pub-date><pmc-comment> PMC Release delay is 0 months and 0 days and was based on the
<volume>7</volume><issue>1</issue><fpage>102</fpage><lpage>108</lpage><permissions><copyright-statement>Copyright © 2014 by S. Karger AG, Basel</copyright-statement><copyright-year>2014</copyright-year><license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by-nc/3.0/"><license-p>This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (www.karger.com/OA-license), applicable to the online version of the article only. 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 http://www.karger.com and the terms of this license are included in any shared versions.</license-p></license></permissions><abstract><p>Here, we present 2 case reports of patients with desmoplastic small round cell tumor (DSRCT), a very rare and aggressive mesenchymal cancer, and we discuss 2therapeutic options for this sarcoma. This report focuses on men aged 22 and 37 years, respectively. The first patient presented with an abdominopelvic mass which was not suitable for surgery. He underwent chemotherapy (adriblastina and cisplatin) with a brief partial remission and survival time of 13 months. The second patient presented with an abdominal mass and underwent partial resection. He received chemotherapy and bevacizumab, resulting in a partial remission and a survival time of 34 months. The extent of surgery and monoclonal antibody use probably had a positive impact on survival. It is necessary to include specific targeted therapies in an attempt to improve survival. Surprisingly, positron emission tomography was not effective in restaging of the second patient, emphasizing the importance of computed tomography or magnetic resonance in DSRCT.</p></abstract><kwd-group><title>Key words</title><kwd>Desmoplastic small round cell tumor</kwd><kwd>Chemotherapy</kwd><kwd>Abdominal tumor</kwd><kwd>Bevacizumab</kwd></kwd-group><counts><fig-count count="3"></fig-count><table-count count="1"></table-count><ref-count count="15"></ref-count><page-count count="7"></page-count></counts></article-meta></front><body><sec id="sec1_1"><title>Introduction</title><p>Desmoplastic small round cell tumor (DSRCT) is a very rare and aggressive mesenchymal cancer, characterized by intraperitoneal masses without an apparent organ of origin [<xref rid="B1" ref-type="bibr">1</xref>]. Metastases are most common to the liver and lungs [<xref rid="B2" ref-type="bibr">2</xref>]. It resembles other small round cell tumors [<xref rid="B3" ref-type="bibr">3</xref>] as described by Gerald and Rosai in 1989 [<xref rid="B4" ref-type="bibr">4</xref>]. There are fewer than 200 reported cases [<xref rid="B2" ref-type="bibr">2</xref>]. It occurs mainly in children, young adults, and males [<xref rid="B5" ref-type="bibr">5</xref>, <xref rid="B6" ref-type="bibr">6</xref>] with an average age of 22 years [<xref rid="B7" ref-type="bibr">7</xref>].</p><p>Histologically, it is characterized by clustering of small round cells separated by desmoplastic stroma [<xref rid="B2" ref-type="bibr">2</xref>]. In immunohistochemistry (IH) there are epithelial, mesenchymal, and positive neuronal markers [<xref rid="B8" ref-type="bibr">8</xref>]. The translocation t(11; 22)(p13; q12) is considered exclusive to DSRCT [<xref rid="B2" ref-type="bibr">2</xref>]. There is no therapeutic pattern for DSRCT [<xref rid="B1" ref-type="bibr">1</xref>]. Chemotherapy and radiotherapy cause temporary improvement and are therefore essential for surgical cytoreduction for increasing survival time.</p></sec><sec id="sec1_2"><title>Case Reports</title><sec id="sec2_1"><title>Case 1</title><p>A 22-year-old male presented with a 3-month history of intensive backaches and weight loss. Later, he noticed a mass in the hypogastrium region and lower limb lymphedema. It evolved into intestinal semiocclusion.</p><p>On computed tomography (CT), performed on August 18, 2009, the mass was confirmed to be a retrovesical tumor (fig. <xref ref-type="fig" rid="F1">1</xref>), extending to the umbilical region, with peritoneal implants and the liver capsule (7.0 cm). A laparoscopy with biopsy was done, confirming an unresectable retroperitoneal mass. Histology and IH studies (table <xref ref-type="table" rid="T1">1</xref>) confirmed the diagnosis of DSRCT.</p><p>On September 19, 2009, with a performance status (PS) of 3, the patient began chemotherapy [doxorubicin 60 mg/m<sup>2</sup> and cisplatin 60 mg/m<sup>2</sup> on day (D)1], as available from the public service. On D15, resolution of the bowel obstruction was noted. After the 3rd cycle of chemotherapy, abdominal CT still showed a solid heterogeneous mass, but with a reduction of 42%. After the 8th cycle of chemotherapy, on May 3, 2010, a new CT scan showed stabilization of the lesions; however, they remained inoperable. The patient was referred for outpatient palliative care. On September 30, 2010, he unfortunately progressed to death due to neoplastic cachexia. Since the initial diagnosis, he had had a survival time of 13 months.</p></sec><sec id="sec2_2"><title>Case 2</title><p>A 37-year-old male presented with a 2-month history of poor digestion and weight loss of 7 kg. On clinical examination, a right abdominal tumor mass was found. Magnetic resonance imaging (MRI) confirmed an abdominal lesion, measuring 18.9 × 16.6 cm, on the periphery of the liver segment VI. A chest CT scan was normal. At exploratory laparotomy, on October 7, 2008, a tumor attached to the liver, invading the splenic hilum, omentum, and mesenteric region was found. Partial resection was possible. Histology and IH studies (table <xref ref-type="table" rid="T1">1</xref>) were compatible with DSRCT.</p><p>He started chemotherapy (carboplatin AUC5, paclitaxel 180 mg/m<sup>2</sup>, and bevacizumab 7.5 mg/kg) on November 18, 2008, for 21/21 days, with a covenant provided by private health insurance. He had PS 3 (73 kg) and changes in liver function. After the 3rd round of chemotherapy, on January 16, 2009, MRI already showed partial remission (78.6% reduction). After the 8th cycle, on May 12, 2009, a presurgical positron emission tomography (PET) reassessment was performed. Interestingly, PET showed physiological distribution of F-18-fluorodeoxyglucose (FDG) without identifying any uptake (fig. <xref ref-type="fig" rid="F2">2</xref>, fig. <xref ref-type="fig" rid="F3">3</xref>). A concomitant CT scan showed tumor infiltration of the hilum, hepatic parenchyma, and peritoneal implants (fig. <xref ref-type="fig" rid="F2">2</xref>).</p><p>A new surgical approach was contraindicated. On June 16, 2009, chemotherapy was stopped at the patient's request. Two months after that, there was a clinical and liver function worsening due to tumor progression and compression of the biliary tract. A retrograde endoscopic cholangiopancreatography was done and a stent was successfully placed.</p><p>Chemotherapy was restarted on August 26, 2009. He already had new partial remission. This scheme was discontinued after the 10th cycle on March 30, 2010 (4.9-cm tumor) due to more frequent occurrences of neutropenia. Chemotherapy was then initiated with cyclophosphamide 50 mg/day, oral, and bevacizumab; this regimen was maintained until November 16, 2010, when it was suspended because of worsening of the patient's clinical state and laboratory findings. He remained in hospice care until his death on July 22, 2011, due to neoplastic cachexia and hepatic encephalopathy. He had had a survival time of 34 months after the initial diagnosis.</p></sec></sec><sec sec-type="discussion" id="sec1_3"><title>Discussion</title><p>We presented 2 cases in men, 22 and 37 years old, respectively, emphasizing that DSRCT occurs predominantly in young males [<xref rid="B2" ref-type="bibr">2</xref>, <xref rid="B7" ref-type="bibr">7</xref>, <xref rid="B9" ref-type="bibr">9</xref>]. There is no known ethnic predisposition [<xref rid="B1" ref-type="bibr">1</xref>]. The common symptoms are abdominal distention or pain, palpable mass, and weight loss [<xref rid="B8" ref-type="bibr">8</xref>] which intensifies with tumoral progression [<xref rid="B2" ref-type="bibr">2</xref>, <xref rid="B10" ref-type="bibr">10</xref>]. Due to unspecific complaints, the diagnosis is always late, compromising the curative treatment, as in the 2 cases presented here, with advanced abdominal disease [<xref rid="B7" ref-type="bibr">7</xref>, <xref rid="B9" ref-type="bibr">9</xref>]. Hepatic metastases are found in 42% of cases, less frequently in the lungs [<xref rid="B1" ref-type="bibr">1</xref>] and rarely in the bones or bone marrow [<xref rid="B10" ref-type="bibr">10</xref>].</p><p>There are no known risks or etiological factors [<xref rid="B1" ref-type="bibr">1</xref>]. DSRCT presents an exclusive translocation, t(11,22)(p13;q12), resulting in EWS and WTI gene fusion [<xref rid="B1" ref-type="bibr">1</xref>, <xref rid="B7" ref-type="bibr">7</xref>, <xref rid="B8" ref-type="bibr">8</xref>, <xref rid="B9" ref-type="bibr">9</xref>, <xref rid="B10" ref-type="bibr">10</xref>, <xref rid="B11" ref-type="bibr">11</xref>, <xref rid="B12" ref-type="bibr">12</xref>, <xref rid="B13" ref-type="bibr">13</xref>, <xref rid="B14" ref-type="bibr">14</xref>], which occurs mainly in the embryonic mesenchymal cells of the serosa of the abdominal organs [<xref rid="B7" ref-type="bibr">7</xref>]. The primary tumor's organ of origin could not be detected in our patients, nor did we do a genetic study since there was conclusive IH, with epithelial, mesenchymal, and some positive neuronal markers [<xref rid="B1" ref-type="bibr">1</xref>, <xref rid="B2" ref-type="bibr">2</xref>, <xref rid="B7" ref-type="bibr">7</xref>, <xref rid="B8" ref-type="bibr">8</xref>, <xref rid="B9" ref-type="bibr">9</xref>, <xref rid="B15" ref-type="bibr">15</xref>]. Histology also confirmed the small rounded cell pattern [<xref rid="B9" ref-type="bibr">9</xref>]. When histology is inconclusive, in up to 50% of cases, IH or genetics might help [<xref rid="B10" ref-type="bibr">10</xref>].</p><p>CT is the most used imaging exam [<xref rid="B1" ref-type="bibr">1</xref>], though it might produce false-positive results [<xref rid="B9" ref-type="bibr">9</xref>]. In case 1, CT was used in the response evaluation and progression. In case 2, both CT and MRI were efficient with respect to diagnosis and follow-up, but PET did not show the tumor with physiological distribution of FDG. New cases are required to confirm this possible characteristic of DSRCT.</p><p>Wide tumoral resection is the prognostic factor that impacts the most on an increased survival rate [<xref rid="B2" ref-type="bibr">2</xref>], with an average of 34 months’ survival time. In an inoperable case, survival time is 14 months on average. Our cases confirm the data: case 1 only underwent biopsy and survived 13 months; case 2 had a larger cytoreduction and survived 34 months. A longer survival time occurs in 15–30% of cases [<xref rid="B3" ref-type="bibr">3</xref>], and up to 18% will be alive in 5 years after undergoing wide surgical resection [<xref rid="B9" ref-type="bibr">9</xref>, <xref rid="B13" ref-type="bibr">13</xref>].</p><p>Due to DSRCT's rarity, there is no standard treatment procedure. The average survival time with polychemotherapy is 32 months [<xref rid="B13" ref-type="bibr">13</xref>], with relapse being the rule [<xref rid="B3" ref-type="bibr">3</xref>]. In well-selected cases, intraperitoneal chemotherapy might be used after surgery with minimal residual disease [<xref rid="B2" ref-type="bibr">2</xref>]. The P6 protocol seems to increase the survival rate [<xref rid="B12" ref-type="bibr">12</xref>], alternating cyclophosphamide, doxorubicin, and vincristine with ifosfamide and etoposide, although it is highly myelotoxic. Case 2, treated with bevacizumab associated with chemotherapy, showed a longer partial remission; however, he had a more extensive surgery.</p></sec><sec sec-type="conclusions" id="sec1_4"><title>Conclusion</title><p>The 2 reports were of men aged 22 and 37 years, respectively. The first patient presented with an abdominopelvic mass which was not suitable for surgery. He underwent chemotherapy (adriblastina and cisplatin) with a brief partial remission and survival time of 13 months. The second patient had an abdominal mass which was partially resected. He received chemotherapy (paclitaxel and carboplatin) and bevacizumab, also with partial remission, but with a survival time of 34 months. PET was not effective in restaging of the second patient, emphasizing the importance of CT or MRI in DSRCT.</p><p>Most patients have nonspecific symptoms, delaying diagnosis, and worsening the prognosis, since there are few effective treatment protocols. It is necessary to include specific targeted therapies in an attempt to improve survival.</p></sec><sec id="sec1_5"><title>Disclosure Statement</title><p>No research support was received for the writing of this paper.</p></sec></body><back><ref-list><title>References</title><ref id="B1"><label>1</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Koniari</surname><given-names>K</given-names></name><name><surname>Mahera</surname><given-names>H</given-names></name><name><surname>Nikolaou</surname><given-names>M</given-names></name><etal></etal></person-group><article-title>Intraabdominal desmoplastic small round cell tumor: report of a case and literature review</article-title><source>Int J Surg Case Rep</source><year>2011</year><volume>2</volume><fpage>293</fpage><lpage>296</lpage><pub-id pub-id-type="pmid">22096758</pub-id></element-citation></ref><ref id="B2"><label>2</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Dufresne</surname><given-names>A</given-names></name><name><surname>Cassier</surname><given-names>P</given-names></name><name><surname>Couraud</surname><given-names>L</given-names></name><etal></etal></person-group><article-title>Desmoplastic small round cell tumor: current management and recent findings</article-title><source>Sarcoma</source><year>2012</year><volume>2012</volume><fpage>714986</fpage><pub-id pub-id-type="pmid">22550424</pub-id></element-citation></ref><ref id="B3"><label>3</label><mixed-citation publication-type="other"><person-group person-group-type="author"><name><surname>Perna</surname><given-names>MJ</given-names></name><name><surname>Streck</surname><given-names>CJ</given-names></name></person-group>: <source>A large solitary desmoplastic small round cell tumor. Annu Sci Meet, Southeastern Surg Congr</source>, Birmingham, AL, 2012.</mixed-citation></ref><ref id="B4"><label>4</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Gerald</surname><given-names>WL</given-names></name><name><surname>Rosai</surname><given-names>J</given-names></name></person-group><article-title>Case 2. Desmoplastic small cell tumor with divergent differentiation</article-title><source>Pediatr Pathol</source><year>1989</year><volume>9</volume><fpage>177</fpage><lpage>183</lpage><pub-id pub-id-type="pmid">2473463</pub-id></element-citation></ref><ref id="B5"><label>5</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Órdoñez</surname><given-names>NG</given-names></name><name><surname>El-Naggar</surname><given-names>AK</given-names></name><name><surname>Ro</surname><given-names>JY</given-names></name><etal></etal></person-group><article-title>Intra-abdominal desmoplastic small cell tumor: a light microscopic, immunocytochemical, ultrastructural, and flow cytometric study</article-title><source>Hum Pathol</source><year>1993</year><volume>24</volume><fpage>850</fpage><lpage>865</lpage><pub-id pub-id-type="pmid">8375856</pub-id></element-citation></ref><ref id="B6"><label>6</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Kretschmar</surname><given-names>CS</given-names></name><name><surname>Colbach</surname><given-names>C</given-names></name><name><surname>Bhan</surname><given-names>I</given-names></name><etal></etal></person-group><article-title>Desmoplastic small cell tumor: a report of three cases and a review of the literature</article-title><source>J Pediat Hematol/Oncol</source><year>1996</year><volume>18</volume><fpage>293</fpage><lpage>298</lpage></element-citation></ref><ref id="B7"><label>7</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Gerald</surname><given-names>WL</given-names></name><name><surname>Ladanyi</surname><given-names>M</given-names></name><name><surname>Alava</surname><given-names>E</given-names></name><etal></etal></person-group><article-title>Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants</article-title><source>J Clin Oncol</source><year>1998</year><volume>16</volume><fpage>3028</fpage><lpage>3036</lpage><pub-id pub-id-type="pmid">9738572</pub-id></element-citation></ref><ref id="B8"><label>8</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Ogata</surname><given-names>DC</given-names></name><name><surname>Tatsugui</surname><given-names>JT</given-names></name><name><surname>Machuca</surname><given-names>TN</given-names></name><etal></etal></person-group><article-title>Desmoplastic round small cell tumor: a case report of a neoplasm of difficult diagnosis</article-title><source>Rev Bras Cancer</source><year>2005</year><volume>51</volume><fpage>263</fpage><lpage>266</lpage></element-citation></ref><ref id="B9"><label>9</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Cao</surname><given-names>L</given-names></name><name><surname>Ni</surname><given-names>J</given-names></name><name><surname>Que</surname><given-names>R</given-names></name><etal></etal></person-group><article-title>Desmoplastic small round cell tumor: a clinical, pathological, and immunohistochemical study of 18 Chinese cases</article-title><source>Int J Surg Pathol</source><year>2008</year><volume>16</volume><fpage>257</fpage><lpage>262</lpage><pub-id pub-id-type="pmid">18573782</pub-id></element-citation></ref><ref id="B10"><label>10</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Philippe-Chomette</surname><given-names>P</given-names></name><name><surname>Kabbara</surname><given-names>N</given-names></name><name><surname>Andre</surname><given-names>N</given-names></name><etal></etal></person-group><article-title>Desmoplastic small round cell tumors with EWS-WT1 fusion transcript in children and young adults</article-title><source>Pediat Blood Cancer</source><year>2012</year><volume>58</volume><fpage>891</fpage><lpage>897</lpage></element-citation></ref><ref id="B11"><label>11</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Torres</surname><given-names>US</given-names></name><name><surname>Ribeiro</surname><given-names>MCA</given-names></name><name><surname>Souza</surname><given-names>AS</given-names></name><etal></etal></person-group><article-title>Abdominal desmoplastic small round cell tumor of childhood: case report</article-title><source>J Bras Patol Med Lab</source><year>2010</year><volume>46</volume><fpage>55</fpage><lpage>59</lpage></element-citation></ref><ref id="B12"><label>12</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Kushner</surname><given-names>BH</given-names></name><name><surname>LaQuaglia</surname><given-names>MP</given-names></name><name><surname>Wollner</surname><given-names>N</given-names></name><etal></etal></person-group><article-title>Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy</article-title><source>J Clin Oncol</source><year>1996</year><volume>14</volume><fpage>1526</fpage><lpage>1531</lpage><pub-id pub-id-type="pmid">8622067</pub-id></element-citation></ref><ref id="B13"><label>13</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Gil</surname><given-names>A</given-names></name><name><surname>Portilla</surname><given-names>AG</given-names></name><name><surname>Brun</surname><given-names>EA</given-names></name><etal></etal></person-group><article-title>Clinical perspective on desmoplastic small round-cell tumor</article-title><source>Oncology</source><year>2004</year><volume>67</volume><fpage>231</fpage><lpage>242</lpage><pub-id pub-id-type="pmid">15557784</pub-id></element-citation></ref><ref id="B14"><label>14</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Mrabti</surname><given-names>H</given-names></name><name><surname>Kaikani</surname><given-names>W</given-names></name><name><surname>Ahbeddou</surname><given-names>N</given-names></name><etal></etal></person-group><article-title>Metastatic desmoplastic small round cell tumor controlled by an anthracycline-based regimen: review of the role of chemotherapy</article-title><source>J Gastrointest Cancer</source><year>2012</year><volume>43</volume><fpage>103</fpage><lpage>109</lpage><pub-id pub-id-type="pmid">21301996</pub-id></element-citation></ref><ref id="B15"><label>15</label><element-citation publication-type="journal"><person-group person-group-type="author"><name><surname>Westphalen</surname><given-names>ACA</given-names></name><name><surname>Ferreira</surname><given-names>JHP</given-names></name><name><surname>Daudt</surname><given-names>AW</given-names></name><etal></etal></person-group><article-title>Intaadbominal desmoplastic small round cell tumor: case report (in Portuguese)</article-title><source>Radiol Bras</source><year>2001</year><volume>34</volume><fpage>299</fpage><lpage>304</lpage></element-citation></ref></ref-list></back><floats-group><fig id="F1" orientation="portrait" position="float"><label>Fig. 1</label><caption><p>Case 1: CT scan showing a retrovesical tumor.</p></caption><graphic xlink:href="cro-0007-0102-g01"></graphic></fig><fig id="F2" orientation="portrait" position="float"><label>Fig. 2</label><caption><p>Case 2: CT scans showing hilum and hepatic parenchyma infiltration (PET without any uptake).</p></caption><graphic xlink:href="cro-0007-0102-g02"></graphic></fig><fig id="F3" orientation="portrait" position="float"><label>Fig. 3</label><caption><p>Case 3: physiological distribution of FDG shown on PET without any uptake.</p></caption><graphic xlink:href="cro-0007-0102-g03"></graphic></fig><table-wrap id="T1" orientation="portrait" position="float"><label>Table 1</label><caption><p>IH studies compatible with DSRCT</p></caption><table frame="hsides" rules="groups"><thead><tr valign="top"><th align="left" rowspan="1" colspan="1">Antibody used</th><th align="left" rowspan="1" colspan="1">Case 1</th><th align="left" rowspan="1" colspan="1">Case 2</th></tr></thead><tbody><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-cytokeratins</td><td align="left" rowspan="1" colspan="1">Positive</td><td align="left" rowspan="1" colspan="1">Positive</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-EMA/E29</td><td align="left" rowspan="1" colspan="1">Positive</td><td align="left" rowspan="1" colspan="1">–</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-desmin/D33</td><td align="left" rowspan="1" colspan="1">Positive</td><td align="left" rowspan="1" colspan="1">Positive</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-NSE</td><td align="left" rowspan="1" colspan="1">Positive</td><td align="left" rowspan="1" colspan="1">–</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-vimentin</td><td align="left" rowspan="1" colspan="1">Positive</td><td align="left" rowspan="1" colspan="1">–</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-gene MIC2 (CD99)</td><td align="left" rowspan="1" colspan="1">Positive</td><td align="left" rowspan="1" colspan="1">Negative</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-chromogranin</td><td align="left" rowspan="1" colspan="1">Negative</td><td align="left" rowspan="1" colspan="1">Negative</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-LCA</td><td align="left" rowspan="1" colspan="1">Negative</td><td align="left" rowspan="1" colspan="1">–</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-protein S100</td><td align="left" rowspan="1" colspan="1">Focal positivity</td><td align="left" rowspan="1" colspan="1">–</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Anti-synaptophysin</td><td align="left" rowspan="1" colspan="1">Negative</td><td align="left" rowspan="1" colspan="1">Negative</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Cytokeratins 8 and 18</td><td align="left" rowspan="1" colspan="1">–</td><td align="left" rowspan="1" colspan="1">Positive</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Enolase</td><td align="left" rowspan="1" colspan="1">–</td><td align="left" rowspan="1" colspan="1">Positive</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">Cytokeratin 20</td><td align="left" rowspan="1" colspan="1">–</td><td align="left" rowspan="1" colspan="1">Negative</td></tr><tr valign="top"><td align="left" rowspan="1" colspan="1">CDX-2</td><td align="left" rowspan="1" colspan="1">–</td><td align="left" rowspan="1" colspan="1">Negative</td></tr></tbody></table></table-wrap></floats-group></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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