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1257) Which of the following is the MOST likely phenotypic profile of angiomyolipomas (AMLs)?

Cytokeratin (CK) positive, S100 positive, smooth muscle actin (SMA) positive, MelanA positive, HMB45 positive, tyrosinase positive CK positive, S100 positive, SMA positive, MelanA positive, HMB45 positive, tyrosinase negative CK positive, S100 positive, SMA negative, MelanA positive, HMB45 positive, tyrosinase positive CK negative, S100 negative, SMA positive, MelanA positive, HMB45 positive, tyrosinase negative CK negative, S100 positive, SMA positive, MelanA positive, HMB45 positive, tyrosinase positive

Show Diagnosis/Topic

• Angiomyolipoma (AML) is a member of the perivascular epithelioid cell (PEC) family of tumors.

• While easy to diagnose when classical, sometimes requires immunohistochemical staining for confirmation.

• PECs express markers of melanocytic differentiation, including HMB45, MelanA, and microphthalmia transcription factor (MiTF).

• Tyrosinase expression is variable, with reported rates varying from 14% to 50%.

• AML is negative for S100 and cytokeratin (CK) expression.

Eble JN: Angiomyolipoma of the kidney. Semin Diagn Pathol 1998;15:21-40.

Stone CH, Lee MW, Amin MB, Yaziji H, Gown AM, Ro JY, Tetu B, Paraf F, Zarbo RJ: Renal angiomyolipoma: further immunophenotypic characterization of an expanding morphologic spectrum. Arch Pathol Lab Med 2001;125:751-758.

Zavala-Pompa A, Folpe AL, Jimenez RE, Lim SD, Cohen C, Eble JN, Amin MB: Immunohistochemical study of microphthalmia transcription factor and tyrosinase in angiomyolipoma of the kidney, renal cell carcinoma, and renal and retroperitoneal sarcomas: comparative evaluation with traditional diagnostic markers. Am J Surg Pathol 2001;25:65-70.

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Anatomic Pathology: Immunopathology

1257) Which of the following is the MOST likely phenotypic profile of angiomyolipomas (AMLs)?

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