Primary alveolar soft part sarcoma of the lung

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 excepti...

Full description

Saved in:
Bibliographic Details
Published in:Journal of Korean medical science 2007, 22(2), , pp.369-372
Main Authors: Kim, Yeong Dae, Lee, Chang Hun, Lee, Min Ki, Jeong, Yeon Joo, Kim, Jee Yeon, Park, Do Youn, Sol, Mee Young
Format: Article
Language:English
Subjects:
Adult
Case Report
Female
Humans
Lung Neoplasms - diagnostic imaging
Lung Neoplasms - pathology
Pulmonary Alveoli - diagnostic imaging
Pulmonary Alveoli - pathology
Radiography
Rare Diseases - diagnostic imaging
Rare Diseases - pathology
Sarcoma - diagnostic imaging
Sarcoma - pathology
Soft Tissue Neoplasms - diagnostic imaging
Soft Tissue Neoplasms - pathology
의학일반
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:1011-8934
1598-6357
DOI:10.3346/jkms.2007.22.2.369