Massive Malignant Epithelioid Angiomyolipoma of the Kidney

Main Article Content

Isaac M. Tessone
Benjamin Lichtbroun
Arnav Srivastava
Alexandra L. Tabakin
Charles F. Polotti
Roman Groisberg
Evita Sadimin
Eric A. Singer
Miral S. Grandhi

Keywords

epithelioid angiomyolipoma, mTOR inhibitor, PEComa, renal tumor, tuberous sclerosis

Abstract

Renal angiomyolipomas (AMLs) are a subset of perivascular epithelioid cell neoplasms (PEComas) that are associated with tuberous sclerosis complex (TSC). Epithelioid angiomyolipomas (EAMLs) are a rare variant of AML with more aggressive propensities. EAMLs with malignant potential can be difficult to distinguish from relatively benign AMLs and other renal tumors. Although there are no established criteria for predicting EAML malignancy, there are proposed histologic parameters that are associated with higher tumor risk. EAML can be treated with surgical resection as well as mTOR inhibitors. Here, we present a unique case of a patient with a 36-cm renal EAML metastatic to the lungs that was treated with complete surgical resection of the primary lesion and mTOR inhibition.

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