The alveolar soft-part sarcoma. A clinically malignant, neuro-endocrine tumor, possibly originating from the tissue composing the paraganglia.

Abstract

The cases of two young women with alveolar soft-part sarcomas (ASPS) in one of the upper extremities are reported. The ASPS is a relatively rare malignant tumor of the soft parts that grows slowly and shows a predilection for the extremities; it occurs most often in young women. The tumor is deceptive in that the clinical impression is benign. So far, the only method of treatment is ample surgical excision. The tumor appears to be scarcely radio-sensitive and primary cytostatic therapy gives very little response. Histogenetically, the tumor is related to the neuro-endocrine paraganglion system. The long-term prognosis of the tumor is unfavorable.