Tuberous sclerosis complex(TSC)is an autosomal dominant hereditary disease that affects multiple organs and systems throughout the body.TSC-associated kidney disease is the leading cause of death in adult TSC patients.This article retrospectively analyzed the characteristics of one TSC-related renal giant angiomy-olipoma(RAML)treated with surgery.The patient,25 years old,was diagnosed with tuberous sclerosis complex in 2000 due to multiple maculopapular rashes on both cheeks.At a regular follow-up in July 2019,imaging exam-inations revealed a tumor in the left lower quadrant with a maximum cross-sectional area of 16 cm×7 cm.Genetic testing showed a loss of heterozygosity in the EX18_41 of TSC2.After the diagnosis was confirmed,open left par-tial nephrectomy was performed,during which multiple tumors were found on the kidney surface and the largest one was located on the ventral side with a diameter of approximately 20 cm.After the renal artery was occluded,kidney tumors were completely enucleate.Postoperative pathological confirmed the diagnosis of angiomyolipoma.This case provides a reference for the treatment of TSC-related renal giant hamartoma.
维普
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