Alveolar soft-part sarcoma of the retroperitoneum

A 33-year-old woman was referred to Iida Municipal Hospital because of left back pain. Computed tomography showed a tumor (17 x 11 x 10 cm) in the left retroperitoneal space. T1- and T2-weighted magnetic resonance imaging showed an inhomogeneous mass with marginal blood vessels. The tumor was resected via lumbar oblique incision with the thoraco-abdominal approach. The tumor weighed 1800 g and consisted of nests of 5-100 large, loosely arranged, polygonal cells, surrounded by capillaries, resembling alveoli. The tumor cells were rich in cytoplasm, containing periodic acid-Schiff- and diastase-positive granules and typical crystals. The pathological diagnosis was alveolar soft-part sarcoma (ASPS). Alveolar soft-part sarcoma is a rare soft-tissue tumor that accounts for approximately 0.5-1% of soft-tissue sarcomas. Such tumors originating in the retroperitoneal space are extremely rare. Herein is reported a case of ASPS of the retroperitoneum with radiological and pathological findings.