Atlas One · Host Biology · Scale 07 — System

DICER1 Syndrome

DICER1 syndrome is caused by germline DICER1 mutations with somatic RNase IIIb hotspot second hits; pleuropulmonary blastoma (PPB), cystic nephroma, ovarian Sertoli-Leydig cell tumors, multinodular goiter; PPB is the sentinel tumor; surveillance from infancy.

Also known as: DICER1 syndrome, DICER1 mutation syndrome, familial PPB, pleuropulmonary blastoma hereditary, DICER1 PPB, DICER1 cystic nephroma, DICER1 SLCT, DICER1 goiter, DICER1 cancer predisposition

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.
dicer1-syndrome

DICER1 Syndrome

DICER1 syndrome is caused by germline DICER1 mutations with somatic RNase IIIb hotspot second hits; pleuropulmonary blastoma (PPB), cystic nephroma, ovarian Sertoli-Leydig cell tumors, multinodular goiter; PPB is the sentinel tumor; surveillance from infancy.

Entry Metadata

FieldValue
IDdicer1-syndrome
NameDICER1 Syndrome
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale07-system

Cross-Atlas Connections

connects-toDicer1 connects-toMycn connects-toRhabdomyosarcoma connects-toOvarian Cancer connects-toKras connects-toThyroid Cancer connects-toWilms Tumor connects-toBloom Syndrome connects-toLung connects-toKidney connects-toMedulloblastoma connects-toLi Fraumeni Syndrome connects-toType Ii Pneumocyte connects-toRetinoblastoma connects-toAtypical Teratoid Rhabdoid Tumor connects-toCervical Cancer connects-toThyroid connects-toFibroblast connects-toP53 connects-toReproductive System connects-toEndocrine System connects-toEye connects-toBrain connects-toCortisol connects-toPhoton connects-toTestosterone connects-toIodine connects-toNeuron connects-toMtor connects-toWt1 connects-toMacrophage connects-toNitrogen connects-toEndothelial Cell connects-toPancreas connects-toAlveolus connects-toIgf 1 connects-toEstrogen connects-toElectron connects-toLiver connects-toLarge Intestine connects-toAdrenal Gland connects-toSmooth Muscle Cell connects-toNeutrophil connects-toT Cytotoxic Cell connects-toRegulatory T Cell connects-toErythrocyte connects-toVegf connects-toEndometrial Cancer connects-toNatural Killer Cell connects-toRespiratory System connects-toNeuroblastoma connects-toNotch connects-toStat3 connects-toCkd connects-toSepsis connects-toAnemia Of Chronic Disease connects-toVenous Thromboembolism connects-toMajor Depressive Disorder connects-toHeart Failure connects-toAspergillus Fumigatus connects-toGeneralized Anxiety Disorder connects-toRenal System connects-toWound Healing connects-toVaricella Zoster Virus connects-toNervous System connects-toMusculoskeletal System connects-toCardiovascular System connects-toDigestive System connects-toGorlin Syndrome connects-toCowden Syndrome connects-toCancer Chemotherapy connects-toBirt Hogg Dube Syndrome connects-toEwing Sarcoma connects-toLung Slice connects-toTargeted Therapy connects-toCarney Complex connects-toGlomerulus connects-toPeutz Jeghers Syndrome connects-toHereditary Breast Ovarian Cancer connects-toRenal Cell Carcinoma connects-toNeuroendocrine Tumors connects-toOsteosarcoma connects-toMyc connects-toPik3Ca connects-toTert connects-toCyclin D1 connects-toHif 1Alpha connects-toEzh2 connects-toActivin A connects-toThyroid Hormones connects-toAkt connects-toRb1 connects-toFgfr connects-toCdkn2A connects-toAndrogen Receptor connects-toCaspase 3 connects-toWnt Beta Catenin connects-toErk1 2 connects-toPten connects-toE2F1 connects-toTgf Beta connects-toKit connects-toBcl 2 connects-toGalectin 3 connects-toSmad4 connects-toJak1 2 connects-toStat1 connects-toCgas Sting connects-toFoxo connects-toCdk4 6 connects-toMdm2 connects-toPerforin connects-toGsk 3B connects-toDnmt3A connects-toS100A8 A9 connects-toAmpk connects-toAutophagy connects-toArid1A connects-toCcr5 connects-toCxcl12 connects-toIl 6 connects-toIl 1B connects-toIl 33 connects-toComplement C3 connects-toMhc Class Ii connects-toIl 2 connects-toPd 1 connects-toHemoglobin connects-toTroponin Complex connects-toXanthine Oxidase connects-toIl 10 connects-toNitric Oxide connects-toIron connects-toIl 4 connects-toIl 13 connects-toProton connects-toKidney connects-toEye connects-toBrain connects-toThyroid connects-toOvarian Cancer connects-toWilms Tumor connects-toIfn Gamma connects-toIl 12 connects-toType I Interferon connects-toIl 5 connects-toIl 17A connects-toIl 23 connects-toT Helper Cell connects-toMast Cell connects-toC5Ar1 connects-toComplement C5 connects-toFactor H connects-toTransferrin connects-toTslp connects-toBradykinin connects-toErythropoietin connects-toHistamine connects-toMelatonin connects-toC1 Esterase Inhibitor connects-toProlactin connects-toOxytocin connects-toVasopressin connects-toSerotonin connects-toSelenium connects-toSodium connects-toMagnesium connects-toCopper connects-toZinc connects-toPotassium connects-toCalcium connects-toPhosphorus connects-toCarbon connects-toChloride connects-toHydrogen connects-toGlp 1 connects-toAngiotensin Ii connects-toRankl connects-toFibronectin connects-toCgrp connects-toCalcitonin connects-toSubstance P connects-toInsulin Receptor connects-toAldosterone connects-toNorepinephrine connects-toAdrenomedullin connects-toBdnf connects-toOsteopontin connects-toEpinephrine connects-toRenin connects-toMyostatin connects-toAngiopoietin connects-toResistin

Sources