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

Sources