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Huge renal angiomyolipomas in tuberous sclerosis complex
Author(s) -
CHAN SY,
CHAN WKY
Publication year - 2005
Publication title -
nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.752
H-Index - 61
eISSN - 1440-1797
pISSN - 1320-5358
DOI - 10.1111/j.1440-1797.2005.00425.x
Subject(s) - tuberous sclerosis , medicine , penetrance , angiomyolipoma , nephrectomy , gross hematuria , tsc2 , kidney , pathology , radiology , biochemistry , chemistry , gene , phenotype , apoptosis , pi3k/akt/mtor pathway
SUMMARY: 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.