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.

Removal of an intra-abdominal desmoplastic small round cell tumor by repetitive debulking surgery: A case report and literature review

“…Histopathologically, the majority of tumors exhibit a nesting or solid/diffuse pattern, with a characteristic histological appearance of high cellularity, undifferentiated small to medium size uniform round cells and a sparse cytoplasm. Nuclei are round to oval shaped and morphologically arranged in nest or spindle cell formation, embedded in dense and metachromatic desmoplastic stroma (13–15). The cells have high nuclear/cytoplasmic ratios with granular chromatin that is reminiscent of small cell carcinoma and pseudorosettes are observed in certain specimens.…”