Key result
Four cases of retroperitoneal synovial sarcoma presented with nonspecific pain or mass, showed typical biphasic histological patterns, and two patients with follow-up died of peritoneal sarcomatosis.
Case Report (n=4)
Retroperitoneal synovial sarcoma is a rare presentation characterized by a biphasic histological pattern and potentially poor prognosis due to peritoneal sarcomatosis.
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Alerts clinicians to include synovial sarcoma in retroperitoneal mass differentials; leaves open optimal diagnostic and therapeutic approaches.
Synovial sarcoma most commonly affects the extremities, especially the lower thigh and knee region; other primary sites, in contrast, have been only infrequently reported. Therefore, we undertook this study of four synovial sarcomas arising in the retroperitoneum. This group, whose ages ranged from 17-57 years (mean, 36 years), comprised three males and one female. The nonspecific clinical presentations consisted of pain and/or a mass; diverse preoperative radiographic procedures merely served to reinforce the impression of a retroperitoneal mass. Histologically, all four examples revealed a typical biphasic pattern. Two cases showed a predominant spindle cell component; two others disclosed a more even distribution of the spindle-cell and epithelioid-cell elements, one of these containing, in addition, broad areas of stromal calcification. Follow-up data, obtained for two patients, indicated that both died as a direct result of peritoneal sarcomatosis. The differential diagnosis of selected spindle-cell and biphasic neoplasms known to arise in the retroperitoneum will be discussed.
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Barry M. Shmookler (1982) conducted a case report in Retroperitoneal synovial sarcoma (n=4). Four cases of retroperitoneal synovial sarcoma presented with nonspecific pain or mass, showed typical biphasic histological patterns, and two patients with follow-up died of peritoneal sarcomatosis.
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