ABSTRACT Tuberous sclerosis is a rare autosomal dominant genetic disorder caused by mutations in tumor suppressor genes (TSC1/2), leading to hamartomas in multiple organs. Renal angiomyolipomas are often asymptomatic but can cause mass effects or bleeding if they enlarge, requiring treatment. A 29‐year‐old woman presented with severe left flank pain, an abdominal mass, and facial papules. Imaging revealed a large, 18.3 × 13 × 11.5 cm mass in the lower pole of the left kidney, which was fat‐poor, a smaller lesion on the opposite kidney, pulmonary cysts, and osteoblastic bone lesions, all indicating tuberous sclerosis‐associated lymphangioleiomyomatosis. A left nephrectomy was performed, and she recovered well. Histology confirmed a renal angiomyolipoma with chronic inflammation. This case underscores the importance of nephrectomy for managing large angiomyolipomas to alleviate symptoms and improve quality of life. Managing such patients requires a multidisciplinary approach, including imaging and genetic testing, with ongoing care and surveillance to achieve better outcomes.
Lema et al. (Thu,) studied this question.