Han-Yu Tsai
Chang Gung Memorial Hospital, Taiwan
Title: Huge retroperitoneal epithelioid angiomyolipoma: A case report
Biography
Biography: Han-Yu Tsai
Abstract
Angiomyolipoma (AML) is a type of tumor in the perivascular epithelioid cell neoplasm (PEComa) family and is the most common benign solid renal neoplasm. Epithelioid angiomyolipoma (EAML), having malignant potential, is considered a rare variant of angiomyolipoma. The most common site of EAMLs is kidney, and extra-renal EAMLs are very uncommon. To our acknowledgment, only six cases of retroperitoneal EAML were reported in the English literature. Presented here is a 46-year-old female with a giant (20 cm×15 cmx15 cm) retroperitoneal epithelioid angiomyolipoma. On pre-OP CT, the tumor was encapsulated with soft tissue and displacing the left kidney upwards and compressing abdominal aorta. The cystic mass was decompressed for 1300 ml of blood and was dissected carefully from the left kidney. Pathology revealed tumor composed largely of polygonal epithelioid cells with abundant eosinophilic cytoplasm, marked nuclear pleomorphism and hyperchromasia, and occasional bizarre tumor giant cells. There were no identifiable renal tissues seen. Hence, the tumor is most likely arising from the renal capsule. Follow-up CT urography and creatinine after three years revealed no evidence of recurrence or metastasis.