Epithelioid angiomyolipoma of the kidney: a case series with literature review

Abstract

OBJECTIVES: To describe four rare cases of epithelioid angiomyolipoma (PEComa) and to discuss the tumour entity and its clinical behavior. PATIENTS AND METHODS: Histopathology reports on partial or total nephrectomy specimens from 2001 to 2016 were reviewed. Patient data, presentation and treatment outcomes on patients with pathological diagnosis of PEComa were reviewed. RESULTS: Three of the patients presented with incidental finding of renal mass, while the fourth patient presented with painful abdominal mass. The first three cases were managed as small renal mass based on CT findings with partial nephrectomy performed. CT of the last case showed large and locally advanced tumour. Radical nephrectomy, right adrenalectomy and wedge resection of the liver were performed. There was no renal biopsy for all 4 cases. The former three patients remained disease-free during subsequent follow-up and the last patient died of liver metastasis in 6 months despite chemotherapy. CONCLUSION: PEComa is a rare entity sharing common presentation as renal tumours, with malignant potential in some of the cases.