J Korean Med Sci.  2007 Apr;22(2):369-372. 10.3346/jkms.2007.22.2.369.

Primary Alveolar Soft Part Sarcoma of the Lung

• Kim YD
• Lee CH
• Lee MK
• Jeong YJ
• Kim JY
• Park DY
• Sol MY

• KMID: 1713204
• DOI: http://doi.org/10.3346/jkms.2007.22.2.369

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.

Keyword

Sarcoma, Alveolar Soft Part; Lung

MeSH Terms

Soft Tissue Neoplasms/pathology/radiography
Sarcoma/*pathology/*radiography
Rare Diseases/pathology/radiography
Pulmonary Alveoli/*pathology/*radiography
Lung Neoplasms/*pathology/*radiography
Humans
Female
Adult

Figure

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  Fig. 1 The computed tomographic scan demonstrates a well-circumscribed tumor located in the posterior segment of the right upper lobe of the lung.

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  Fig. 2 The tumor shows characteristic alveolar nests of large polygonal tumor cells. Emphysematous lung tissue is found in the left upper corner, and arrows indicate bronchioles entrapped within the tumor mass (hematoxylin-eosin, ×200).

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  Fig. 3 Periodic acid-Schiff preparation with diastase digestion highlights the distinctive intracytoplasmic granules or rod-like structures in the cytoplasm of the tumor cells (D-PAS, ×400).

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  Fig. 4 Immunohistochemical stain for alpha-smooth muscle actin demonstrates a focally positive reaction in the tumor (SMA immunostain, ×400).

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  Fig. 5 Electron microphotograph shows electron-dense rhomboid intracytoplasmic crystals in the cytoplasm of the tumor cells (×8,000).

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  Fig. 6 Metastatic alveolar soft part sarcoma involving the lumbar vertebrae shows the same histologic features as the primary lung tumor. An asterisk indicates a piece of destroyed bony spicule (hematoxylin-eosin, ×200).

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