Pulmonary angiomyolipoma recurring 26 years after nephrectomy for angiomyolipoma: benign clinical course

Angiomyolipoma (AML), a benign renal tumor, has various clinical forms, and the nature of AML is not sufficiently understood because of few reports of long-term observation. We report a 57-year-old female without tuberous sclerosis who developed multiple pulmonary AML 26 years after a right nephrectomy for renal AML. A computed tomogram demonstrated multifocal round lesions with lipid-like density throughout both lung fields. An open lung biopsy revealed a histological diagnosis of pulmonary AML. An abdominal computed tomogram was also compatible with splenic AML. She has been in good health for 12 years since the lung biopsy over the 38 years since the nephrectomy. This case suggests that multicentric AML can recur at distant organs even after long-term silence in a patient who has a past history of renal AML.