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DICER1-related cancer predisposition
Gene: DICER1
DICER1 (dicer 1, ribonuclease III)
EnsemblGeneIds (GRCh38): ENSG00000100697
EnsemblGeneIds (GRCh37): ENSG00000100697
OMIM: 606241, Gene2Phenotype
DICER1 is in 11 panels
EnsemblGeneIds (GRCh38): ENSG00000100697
EnsemblGeneIds (GRCh37): ENSG00000100697
OMIM: 606241, Gene2Phenotype
DICER1 is in 11 panels
1 review
Achchuthan Shanmugasundram (Genomics England Curator)
Comment on phenotypes: This gene has been associated with relevant phenotypes in OMIM (MIMs #138800 & #601200) and the OMIM records were last accessed on 17 December 2025.Created: 17 Dec 2025, 11:39 p.m. | Last Modified: 17 Dec 2025, 11:39 p.m.
Panel Version: 1.2
DICER1 has been added to the panel for R364 DICER1-related cancer predisposition with a green rating as agreed with the NHS Genomic Medicine Service.Created: 30 Jun 2023, 11:55 a.m. | Last Modified: 30 Jun 2023, 11:55 a.m.
Panel Version: 0.1
Mode of inheritance
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Details
- Mode of Inheritance
- MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
- Sources
-
- NHS GMS
- Expert Review Green
- Phenotypes
-
- Goiter, multinodular 1, with or without Sertoli-Leydig cell tumors, OMIM:138800
- Pleuropulmonary blastoma, OMIM:601200
- DICER1-related tumor predisposition, MONDO:0100216
- OMIM
- 606241
- Clinvar variants
- Variants in DICER1
- Penetrance
- None
- Panels with this gene
-
- Sarcoma susceptibility
- DICER1-related cancer predisposition
- Childhood solid tumours
- Adult solid tumours cancer susceptibility
- Sarcoma cancer susceptibility
- Inherited non-medullary thyroid cancer
- Fetal anomalies
- COVID-19 research
- Familial rhabdomyosarcoma
- Childhood solid tumours cancer susceptibility
- Adult solid tumours for rare disease
History Filter Activity
17 Dec 2025, Gel status: 3
Set Phenotypes
Achchuthan Shanmugasundram (Genomics England Curator)Phenotypes for gene: DICER1 were changed from to Goiter, multinodular 1, with or without Sertoli-Leydig cell tumors, OMIM:138800; Pleuropulmonary blastoma, OMIM:601200; DICER1-related tumor predisposition, MONDO:0100216
29 Jun 2023, Gel status: 3
Created, Added New Source, Set mode of inheritance
Achchuthan Shanmugasundram (Genomics England Curator)gene: DICER1 was added gene: DICER1 was added to DICER1-related cancer predisposition. Sources: Expert Review Green,NHS GMS Mode of inheritance for gene: DICER1 was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted