Literature DB >> 9149753

Disseminated intrathoracic desmoplastic small round-cell tumor: a case report.

L Venkateswaran1, J J Jenkins, S C Kaste, S A Shurtleff, J R Downing, A S Pappo.   

Abstract

PURPOSE: Recently recognized as a distinct clinicopathologic entity, desmoplastic small round-cell tumors typically affect young men. These aggressive tumors usually arise in the abdomen; other sites of primary disease have been described only rarely. We report the case of an extraabdominal primary tumor with widespread dissemination, including the subcutaneous tissue, a previously unrecognized metastatic site. PATIENT AND METHODS: We describe the case of a 16-year-old boy with a primary extraabdominal metastatic desmoplastic small round-cell tumor.
RESULTS: Our patient had a primary intrathoracic desmoplastic small round-cell tumor and widespread dissemination involving the subcutaneous tissue, kidney, liver, bone, and lymph nodes. Histopathologic analysis found intense desmoplasia and polyphenotypic expression of neural, muscle, and epithelial markers. Reverse transcriptase-polymerase chain reaction analysis of fresh tumor tissue confirmed the characteristic EWS-WT1 transcript.
CONCLUSIONS: Broader than originally anticipated, the clinical spectrum of desmoplastic small round-cell tumors continues to evolve. Primary intrathoracic tumors with soft-tissue dissemination and polyphenotypic expression should prompt suspicion of this malignancy. Molecular analysis of fresh tumor tissue is an important adjunct to diagnosing this rare neoplasm.

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Year:  1997        PMID: 9149753     DOI: 10.1097/00043426-199703000-00017

Source DB:  PubMed          Journal:  J Pediatr Hematol Oncol        ISSN: 1077-4114            Impact factor:   1.289


  5 in total

1.  Combined resection and multi-agent adjuvant chemotherapy for desmoplastic small round cell tumor arising in the abdominal cavity: report of a case.

Authors:  Chang-Cheng Chang; Jun-Te Hsu; Jeng-Hwei Tseng; Tsann-Long Hwang; Han-Ming Chen; Yi-Yin Jan
Journal:  World J Gastroenterol       Date:  2006-02-07       Impact factor: 5.742

2.  Characteristic imaging features of desmoplastic small round cell tumour.

Authors:  Vandan Caur Arora; Anita P Price; Stephen Fleming; Michael J Sohn; Heather Magnan; Michael P LaQuaglia; Sara Abramson
Journal:  Pediatr Radiol       Date:  2012-11-23

3.  Desmoplastic small round cell tumor of the middle ear: A case report.

Authors:  Jing Xu; Mengwei Yao; Xinxin Yang; Tao Liu; Shaohua Wang; Dengdian Ma; Xiaoyu Li
Journal:  Medicine (Baltimore)       Date:  2018-04       Impact factor: 1.889

4.  Desmoplastic small round cell tumor of stomach.

Authors:  Ahmed Abu-Zaid; Ayman Azzam; Asma Alnajjar; Hussa Al-Hussaini; Tarek Amin
Journal:  Case Rep Gastrointest Med       Date:  2013-06-06

Review 5.  Desmoplastic small round cell tumors of the pleura: a review of the clinical literature.

Authors:  Alessandro Giuseppe Fois; Pietro Pirina; Antonella Arcadu; Francesca Becciu; Sandra Manca; Viviana Marras; Sara Canu; Gaetano Castagna; Giorgio Carlo Ginesu; Angelo Zinellu; Panagiotis Paliogiannis
Journal:  Multidiscip Respir Med       Date:  2017-09-09
  5 in total

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