Primary Alveolar Soft Part Sarcoma (ASPS) of the Breast

Author:

Wu Julie1,Brinker David A.,Haas Mark,Montgomery Elizabeth A.1,Argani Pedram2

Affiliation:

1. Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore; Department of Pathology, St. Joseph's Medical Center, Towson, MD.

2. Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore; Department of Pathology, St. Joseph's Medical Center, Towson, MD; The Johns Hopkins Hospital, Surgical Pathology, Weinberg Building, Room 2242, 401 N. Broadway, Baltimore, Maryland 21231-2410.

Abstract

Alveolar soft part sarcoma (ASPS) is a rare neoplasm that most commonly presents in the lower extremities. Although ASPS has distinctive histologic features, it may cause diagnostic problems when it arises in unusual locations. To our knowledge, only 1 case of ASPS arising within the breast has previously been reported. Here, we report a second case of primary mammary ASPS. The patient was a 44-year-old woman who presented with a breast mass. Needle biopsy was performed, yielding a polygonal cell lesion with abundant, predominantly xanthomatous cytoplasm. The cells labeled strongly for the histiocytic marker CD68, suggesting a benign macrophage-rich lesion. However, the unusual nature of the lesion as well as the prominence of nucleoli prompted suggestion for an excision. The excision more clearly revealed the lesion's alveolar architecture and demonstrated cells with more eosinophilic cytoplasm, along with the xanthomatous cells. The diagnosis of ASPS was confirmed by electron microscopy, which revealed characteristic membranebound rhomboidal crystals, as well as by nuclear labeling for TFE3 protein by immunohistochemistry. With this report, we confirm the utility of a novel immunohistochmical technique for the identification of an ASPS presenting in an unusual locale.

Publisher

SAGE Publications

Subject

Pathology and Forensic Medicine,Surgery,Anatomy

Reference11 articles.

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3. Alveolar soft-part sarcoma. A clinico-pathologic study of half a century

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