
Type 2 papillary kidney cancer. Hereditary leiomyomatosis renal cell carcinoma (HLRCC) is an autosomal dominant hereditary cancer syndrome (right lower panel) in which affected individuals are at risk for the development of type 2 kidney cancer (upper left and upper middle panels), cutaneous leiomyomas (right upper, left lower, and middle lower panels) and early onset uterine leiomyomas (fibroids). HLRCC is characterized by germline mutation of the gene for the Krebs cycle enzyme, fumarate hydratase. (Fig. 5 from Linehan et al. 2003.)











