Primary Pulmonary Alveolar Soft-part Sarcoma

Abstract

Alveolar soft-part sarcoma rarely arises in the lung. Only two patients with tumors that were apparently primary in the lung have been documented in the literature. We report an additional case in a 25-year-old woman. The tumor formed a solitary 5 cm, solid mass in the left perihilar region. Clinical examination, radiologic studies, and follow-up examination of more than 1 year have failed to reveal tumor in an extrapul monary location. The light microscopic appearance was typical for alveolar soft-part sarcoma, and crystalloids were identified in the tumor cells by the periodic acid-Schiff stain and by electron microscopy, confirming the diagnosis. Immunocytochemical studies did not show evidence of myogenic, epithelial, or neuroendocrine differentia tion. Int J Surg Pathol 2(1):57-62, 1994