Atlas One · Host Biology · Scale 07 — System

Hereditary Leiomyomatosis and Renal Cell Carcinoma

Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is caused by germline FH mutations; cutaneous and uterine leiomyomas + aggressive FH-deficient RCC; collecting duct-like histology; fumarate drives HIF-1α + immune evasion; bevacizumab + erlotinib standard.

Also known as: HLRCC, hereditary leiomyomatosis renal cell carcinoma, FH syndrome, Reed syndrome, FH-deficient RCC, HLRCC RCC, FH leiomyoma, fumarate hydratase deficiency, FH hereditary cancer, leiomyomatosis RCC

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
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hlrcc

Hereditary Leiomyomatosis and Renal Cell Carcinoma

Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is caused by germline FH mutations; cutaneous and uterine leiomyomas + aggressive FH-deficient RCC; collecting duct-like histology; fumarate drives HIF-1α + immune evasion; bevacizumab + erlotinib standard.

Entry Metadata

FieldValue
IDhlrcc
NameHereditary Leiomyomatosis and Renal Cell Carcinoma
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toFh connects-toHif 1Alpha connects-toVhl connects-toRenal Cell Carcinoma connects-toSmooth Muscle Cell connects-toVegf connects-toKidney connects-toReproductive System connects-toSkin connects-toPheochromocytoma Paraganglioma

Sources