The morphologic spectrum of kidney tumors in hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome

MJ Merino, C Torres-Cabala, P Pinto… - The American journal …, 2007 - journals.lww.com
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is an autosomal dominant
familial syndrome characterized by the development of cutaneous and uterine leiomyomas
as well as renal tumors. The mutation of this condition has been identified in the fumarate
hydratase (FH, 1q42. 3-q43) gene. The histology of the renal cancers has not been well
described or illustrated because of the newness of the syndrome. We reviewed 40 renal
tumors resected from 38 patients belonging to HLRCC families with proven fumarate …