Primary Alveolar Soft Part Sarcoma of the Lung.
10.3346/jkms.2007.22.2.369
- Author:
Yeong Dae KIM
1
;
Chang Hun LEE
;
Min Ki LEE
;
Yeon Joo JEONG
;
Jee Yeon KIM
;
Do Youn PARK
;
Mee Young SOL
Author Information
1. Department of Thoracic & Cardiovascular Surgery, College of Medicine, Pusan National University, Busan, Korea.
- Publication Type:Case Report ; Research Support, Non-U.S. Gov't
- Keywords:
Sarcoma, Alveolar Soft Part;
Lung
- MeSH:
Soft Tissue Neoplasms/pathology/radiography;
Sarcoma/*pathology/*radiography;
Rare Diseases/pathology/radiography;
Pulmonary Alveoli/*pathology/*radiography;
Lung Neoplasms/*pathology/*radiography;
Humans;
Female;
Adult
- From:Journal of Korean Medical Science
2007;22(2):369-372
- CountryRepublic of Korea
- Language:English
-
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. Ultrastuctural study using electron microscopy revealed characteristic electron-dense, rhomboid intracytoplasmic crystals.
