Huge renal angiomyolipomas in tuberous sclerosis complex.
Tuberous sclerosis complex (TSC) is an autosomal dominance disorder with variable penetrance. Renal angiomyolipoma (AML) is one of the commonest urological manifestations. These lesions may cause significant morbidity and mortality. We report two patients with multiple and huge renal angiomyolipomas presenting with gross haematuria. They subsequently underwent unilateral nephrectomy. The difficulty in diagnosis and controversies in management will be briefly discussed.
Chan
S Y
SY
Department of Paediatrics, Queen Elizabeth Hospital, Hong Kong SAR.
Chan
W K Y
WK
D000293:Adolescent; D018207:Angiomyolipoma / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D000951:Antigens, Neoplasm; D002648:Child; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins / Q000032:analysis; D014402:Tuberous Sclerosis / Q000150:complications*; D000077004:Tuberous Sclerosis Complex 1 Protein; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins / Q000235:genetics
D000951:Antigens, Neoplasm; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins; D000077004:Tuberous Sclerosis Complex 1 Protein; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins