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
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
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 connects-toVhl Disease connects-toTuberous Sclerosis Complex connects-toErythrocyte connects-toBirt Hogg Dube Syndrome connects-toEndometrial Cancer connects-toCowden Syndrome connects-toOxygen connects-toMtor connects-toP53 connects-toIntegumentary System connects-toCarbon connects-toImmune System connects-toIdh Mutant Glioma connects-toLung connects-toCollagen connects-toNfe2L2 connects-toAtp connects-toNatural Killer Cell connects-toAkt connects-toMacrophage connects-toDendritic Cell connects-toAdrenal Gland connects-toIron connects-toEndothelial Cell connects-toPhoton connects-toFibrosis connects-toBrain connects-toElectron connects-toBone Marrow connects-toLiver connects-toAntibody connects-toNeuron connects-toLarge Intestine connects-toErythropoietin connects-toMet connects-toT Cytotoxic Cell connects-toFibroblast connects-toAmpk connects-toNeurofibromatosis Type 1 connects-toSdhb connects-toIron Deficiency Anemia connects-toVenous Thromboembolism connects-toNf Kb connects-toCkd connects-toSepsis connects-toAnemia Of Chronic Disease connects-toHeart Failure connects-toMajor Depressive Disorder connects-toNeuropathic Pain connects-toWound Healing connects-toGeneralized Anxiety Disorder connects-toMusculoskeletal System connects-toEndocrine System connects-toRenal System connects-toRespiratory System connects-toLymphatic System connects-toCardiovascular System connects-toTargeted Therapy connects-toNervous System connects-toDigestive System connects-toCheckpoint Inhibitors connects-toPolycythemia Vera connects-toMen1 Syndrome connects-toGist connects-toSulforaphane connects-toNsclc connects-toCholangiocarcinoma connects-toAlveolus connects-toPeripheral Nerve connects-toArterial Wall connects-toCortical Bone connects-toHepatic Lobule connects-toTet2 connects-toIdh1 connects-toEzh2 connects-toMyc connects-toCyclin D1 connects-toTert connects-toEgln1 connects-toErk1 2 connects-toCcl2 connects-toRad51 connects-toCxcl12 connects-toCaspase 3 connects-toDnmt3A connects-toEgfr connects-toEstrogen connects-toPik3Ca connects-toE2F1 connects-toCdkn2A connects-toPdgf connects-toKras connects-toRb1 connects-toEpas1 connects-toGalectin 3 connects-toPten connects-toStat1 connects-toCgas Sting connects-toCdk4 6 connects-toFoxo connects-toPerforin connects-toMdm2 connects-toGsk 3B connects-toAutophagy connects-toS100A8 A9 connects-toCcr5 connects-toArid1A connects-toSrc Kinase connects-toIl 1B connects-toIl 33 connects-toIl 6 connects-toIl 17A connects-toComplement C3 connects-toCalcineurin connects-toProgesterone connects-toMhc Class Ii connects-toIl 2 connects-toHemoglobin connects-toNitric Oxide connects-toXanthine Oxidase connects-toIl 10 connects-toIl 4 connects-toProstaglandins connects-toIl 13 connects-toBirt Hogg Dube Syndrome connects-toIron connects-toLeptin connects-toAdiponectin connects-toResistin connects-toType I Interferon connects-toIfn Gamma connects-toNeutrophil connects-toIl 12 connects-toIl 23 connects-toIl 5 connects-toIge connects-toMast Cell connects-toT Helper Cell connects-toB Cell connects-toC5Ar1 connects-toTransferrin connects-toComplement C5 connects-toFactor H connects-toTgf Beta connects-toTslp connects-toBradykinin connects-toC1 Esterase Inhibitor connects-toHistamine connects-toMelatonin connects-toTestosterone connects-toProlactin connects-toOxytocin connects-toVasopressin connects-toSerotonin connects-toSelenium connects-toIodine connects-toSodium connects-toMagnesium connects-toCopper connects-toChloride connects-toNitrogen connects-toPotassium connects-toCalcium connects-toHydrogen connects-toPhosphorus connects-toPd 1 connects-toGlp 1 connects-toAngiotensin Ii connects-toWnt Beta Catenin connects-toRankl connects-toSmad4 connects-toFibronectin connects-toNotch connects-toIgf 1 connects-toActivin A connects-toCgrp connects-toCalcitonin connects-toSubstance P connects-toInsulin Receptor connects-toAldosterone connects-toAndrogen Receptor connects-toNorepinephrine connects-toAdrenomedullin

Sources