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William Gerald
 Updated on January 28th, 1998.

"Intra-Abdominal Desmoplastic Small Round-Cell Tumor"


by William Gerald, et al.

Nineteen cases were collected in a study via consultation files of the authors. Clinical manifestations included a predilection of this condition in males, i.e., about 16 out of 19 patients were males. Mean age was 18.6 years, and 17 out of 19 patients were 30 or younger at diagnosis. Symptoms included abdominal distention and pain (abdominal and back), anorexia, weakness, abdominal fullness, and constipation. The physical examination most prominently included a palpable abdominal mass. Laboratory data were non-specific. The diagnosis was obtained by surgical exploration which usually revealed a large intra-abdominal mass with smaller implants anywhere along the peritoneum, especially the pelvis. The treatment consisted of debulking the mass, but the surgeon did not feel that the entire lesion was resected. The debulking was usually followed by multi-drug chemotherapy and occasionally radiation.

Microscopic analysis included the following familiar features: sharply outlined clusters separated by a stroma, i.e., desmoplastic, overall staining was deeply basophilic, central necrosis and calcifications. The tumor cell were uniform. The growth pattern showed an invasive process. In nearly all tested cases, the tumor cells were positive for keratin, epithelial membrane antigen (ema), neuron-specific enolase (nse), vimentin and desmin. This tumor is proposed as another member of the small round blue cell tumors seen in infancy and childhood that allows a multidirectional phenotypic direction.



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