Expression of KIT (CD117) in angiomyolipoma

The American Journal of Surgical Pathology
Hala R MakhloufKamal G Ishak

Abstract

Angiomyolipoma is a unique mesenchymal tumor postulated to arise from perivascular epithelioid cells. Immunohistochemical studies have shown that angiomyolipomas express the melanocytic markers HMB-45, MART-1 (Melan A), microphthalmia transcription factor, and tyrosinase, in addition to smooth muscle actin. KIT (CD117) is a transmembrane growth factor receptor expressed in cells of melanocytic and a variety of other cell lineages. To date, KIT immunoreactivity has not been systematically studied in angiomyolipoma. In this study we immunohistochemically analyzed a series of 21 angiomyolipomas (15 hepatic, six renal) with KIT. All were KIT positive: 14 of 21 (67%) with 3+ staining (>50% of tumor cells), 4 of 21 (19%) with 2+ staining (25-50% of tumor cells), and 3 of 21 (14%) with 1+ staining (<25% of tumor cells). In comparison, the percent of angiomyolipomas showing 3+ staining with HMB-45 was 62% and with Melan A was 52%. Positive KIT staining was detected in the epithelioid, spindle, and intermediate small round cells. Most cases showed diffuse cytoplasmic positivity. Strong perinuclear staining was present in the vacuolated clear epithelioid cells. There was focal KIT staining of fat cells. KIT was not detected in the endoth...Continue Reading

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