title: Primary alveolar soft part sarcoma of the lung.
abstract: Alveolar soft part sarcoma (ASPS) is a rare epithelial-like soft tissue sarcoma. The two main sites of its occurrence are the lower extremities in adults and the head and neck in children. Primary pulmonary involvement of this sarcoma, without evidence of soft tissue tumor elsewhere, is very exceptional. We present a case of primary ASPS of the lung in a 42-yr-old woman. A computed tomographic scan of the thorax demonstrated a well-circumscribed, solid tumor located in the right upper lobe. The mass was resected by right upper lobectomy. After 5 months, three metastatic lesions, involving lumbar vertebrae and occipital scalp, were found. Histologically, the tumor consisted of alveolar nests of large polygonal tumor cells, the cytoplasm of which frequently revealed periodic acid-Schiff-positive, diastase-resistant intracytoplasmic rod-like structures. On immunohistochemical staining, the tumor cells were positive only for vimentin and alpha-smooth muscle actin. Ultrastructural study using electron microscopy revealed characteristic electron-dense, rhomboid intracytoplasmic crystals.
authors: Department of Thoracic & Cardiovascular Surgery, College of Medicine, Pusan National University, Busan, Korea.
authors: Yeong Dae
authors: YD
authors: Kim
authors: Chang Hun
authors: CH
authors: Lee
authors: Min Ki
authors: MK
authors: Lee
authors: Yeon Joo
authors: YJ
authors: Jeong
authors: Jee Yeon
authors: JY
authors: Kim
authors: Do Youn
authors: DY
authors: Park
authors: Mee Young
authors: MY
authors: Sol
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology*; D011650:Pulmonary Alveoli / Q000000981:diagnostic imaging* / Q000473:pathology*; D011859:Radiography; D035583:Rare Diseases / Q000000981:diagnostic imaging / Q000473:pathology; D012509:Sarcoma / Q000000981:diagnostic imaging* / Q000473:pathology*; D012983:Soft Tissue Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology