A. Epithelioid angiomyolipoma
This patient has metastatic epithelioid angiomyolipoma (EAML), an unusual variant of AML, which is among the most commonly encountered types of kidney masses. The resected masses were consistent with an epithelioid mesenchymal neoplasm with clear cell morphology having low to intermediate grade features (low mitotic rate with focal tumor necrosis; Figure 2). The diagnosis of EAML was confirmed with HMB45 and Melan-A staining.
Perivascular epithelioid cell neoplasms (PEComas) is a family of related mesenchymal tumors, including AML, lymphangiomyomatosis, clear cell tumors of the lung, and rare morphologically and immunophenotypically similar tumors.1 These tumors are defined by perivascular epithelioid cells or spindle cells with clear eosinophilic cytoplasm and are typically arranged around blood vessels with immunoreactivity to smooth muscle and melanocytic (HMB5 and Melan-A) markers.2 Typically, PEComa tumors occur at visceral, retroperitoneal, and abdominopelvic sites (the uterus being the most common site)3 and are rarely malignant (only 6% to 7% demonstrate distant metastasis).1