title: 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.
authors.affiliation: Hematology-Oncology Department, Staten Island University Hospital, 256 Mason Avenue, Staten Island, NY 10305, USA. walidbazmd@hotmail.com
authors.forename: Walid
authors.initials: W
authors.lastname: Baz
authors.forename: Raymond
authors.initials: R
authors.lastname: El-Soueidi
authors.forename: Fadi
authors.initials: F
authors.lastname: Nakhl
authors.forename: Nelly
authors.initials: N
authors.lastname: Aoun
authors.forename: Nena
authors.initials: N
authors.lastname: Chin
authors.forename: Meeko
authors.initials: M
authors.lastname: Dhar
mesh_terms: D015085:2,4,5-Trichlorophenoxyacetic Acid / Q000009:adverse effects*; D015084:2,4-Dichlorophenoxyacetic Acid / Q000009:adverse effects*; D000008:Abdominal Neoplasms / Q000139:chemically induced* / Q000473:pathology; D000075182:Agent Orange; D003678:Defoliants, Chemical / Q000009:adverse effects*; D006801:Humans; D008297:Male; D008875:Middle Aged; D000072317:Polychlorinated Dibenzodioxins / Q000009:adverse effects*; D018228:Sarcoma, Small Cell / Q000139:chemically induced* / Q000473:pathology; D014728:Veterans; D047829:Vietnam Conflict
chemical_list: D003678:Defoliants, Chemical; D000072317:Polychlorinated Dibenzodioxins; D015084:2,4-Dichlorophenoxyacetic Acid; D000075182:Agent Orange; D015085:2,4,5-Trichlorophenoxyacetic Acid