Renal epithelioid angiomyolipoma: A case report.

Abstract

Epithelioid angiomyolipoma (EAML) of the kidney is an uncommon neoplasm with malignant potential. It can occur sporadically or be associated with tuberous sclerosis. EAML is a monotypic variant of angiomyolipoma (AML), which is classified as neoplasm of the perivascular epithelioid cell or perivascular epithelioid cell tumor. Due to its epithelioid nature and paucity of fat components, unlike classic AML, which has abundant adipose tissue with characteristic features on CT scans, it is difficult to distinguish EAML from renal cell carcinoma and fat-poor AML on CT or MRI preoperatively, which may lead to misdiagnosis and unnecessary nephrectomy. The present report describes two cases of renal EAML, which were successfully treated by laparoscopic surgery. Preoperative diagnosis had not been achieved until surgery was performed and histological analysis was accomplished. No local recurrence or distal metastasis was observed during follow-up. Although the differential diagnosis was challenging preoperatively, a diagnosis of EAML should be considered and surgical excision was the preferred treatment strategy for the patients with localized tumors.