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Desmoplastic small round-cell tumor: an adult with previous exposure to agent orange.

Abstract
Desmoplastic small round-cell tumor is an uncommon, highly aggressive tumor with a predilection for pediatric age groups and young adults. It is very unusual in the elderly population. Although Agent Orange has been associated with soft-tissue sarcoma, an association with desmoplastic small round-cell tumor has not been reported. A 52-year-old male presented with abdominal distention, dyspnea, and a 9 kg weight loss. Prior history was significant for hepatitis C and diabetes. He was a Vietnam veteran and he admitted being exposed to Agent Orange. On physical examination, the abdomen was distended and tense. Computed tomography scan of the chest, abdomen and pelvis demonstrated extensive mediastinal and retroperitoneal adenopathy, diffuse omental masses and extensive pleural, intra-abdominal and pelvic ascites. Omental core needle biopsy was consistent with desmoplastic small round-cell tumor based on morphology and immunohistochemistry. He responded poorly to chemotherapy with high-dose cyclophosphamide, doxorubicin and vincristine and died 5 months after presentation secondary to neutropenic sepsis despite G-CSF support and antibiotics.
AuthorsWalid Baz, Raymond El-Soueidi, Fadi Nakhl, Nelly Aoun, Nena Chin, Meeko Dhar
JournalJapanese journal of clinical oncology (Jpn J Clin Oncol) Vol. 40 Issue 6 Pg. 593-5 (Jun 2010) ISSN: 1465-3621 [Electronic] England
PMID20382635 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Defoliants, Chemical
  • Polychlorinated Dibenzodioxins
  • 2,4-Dichlorophenoxyacetic Acid
  • Agent Orange
  • 2,4,5-Trichlorophenoxyacetic Acid
Topics
  • 2,4,5-Trichlorophenoxyacetic Acid (adverse effects)
  • 2,4-Dichlorophenoxyacetic Acid (adverse effects)
  • Abdominal Neoplasms (chemically induced, pathology)
  • Agent Orange
  • Defoliants, Chemical (adverse effects)
  • Humans
  • Male
  • Middle Aged
  • Polychlorinated Dibenzodioxins (adverse effects)
  • Sarcoma, Small Cell (chemically induced, pathology)
  • Veterans
  • Vietnam Conflict

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