PMID: 8597865Sep 1, 1995Paper

Intra-abdominal desmoplastic small round cell tumor with extensive extra-abdominal involvement

Pediatric Pathology & Laboratory Medicine : Journal of the Society for Pediatric Pathology, Affiliated with the International Paediatric Pathology Association
M B Resnick, M Donovan

Abstract

Intra-abdominal desmoplastic small round cell tumor (DSRCT) is a recently described, rare neoplasm that usually occurs in male adolescents, has a poor prognosis, and displays a typical clinicopathological profile. This aggressive neoplasm exhibits a predominantly intra-abdominal serosal pattern, with frequent pelvic extension, less frequent retroperitoneal involvement, and rare pulmonary and mediastinal spread. We report on the autopsy findings of a 9-year-old male who presented with a biopsy-proven DSRCT involving the abdomen, retroperitoneum, and scrotum and additional radiologic studies suggesting vertebral, hepatic, and mediastinal involvement. Postmortem examination performed 10 months after presentation confirmed the initial radiologic findings and demonstrated extensive pleural and pulmonary metastases and, for the first time, direct evidence of bone marrow involvement. This tumor exhibited the typical immunophenotype previously reported for DSRCT, namely multidirectional expression of epithelial, neural, and muscular markers.

References

Mar 1, 1992·The American Journal of Surgical Pathology·J PratF Algaba
Jul 1, 1990·The American Journal of Surgical Pathology·F Gonzalez-CrussiC C Sun
Jan 1, 1989·Pediatric Pathology·W L Gerald, J Rosai

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Citations

Aug 26, 1998·Annals of Surgical Oncology·R E SchwarzM P La Quaglia
Sep 25, 1997·The Journal of Urology·J FurmanA D Berry
May 29, 2004·The American Journal of Surgical Pathology·Tarek A BismarN Volkan Adsay
May 1, 2001·International Journal of Urology : Official Journal of the Japanese Urological Association·N MurosakiA Okuyama
Sep 17, 2004·The American Journal of Surgical Pathology·Min-Cheng SuYuan-Chung Chu
Aug 26, 2006·Pathology International·Minoru HamazakiTaemi Nara
Nov 11, 1999·The American Journal of Surgical Pathology·V AdsayJ Rosai
Dec 2, 1998·Australasian Radiology·T H TanY M Au

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