Gene

DICER1

dicer 1, ribonuclease III

Explore in graph →CI-GENE-00043379HGNC:17098 ENSG00000100697 NCBI 23405 CIViC Cancer geneApproved

Curated evidence

Clinical evidence (32)

CIViC items involving this gene, grouped by molecular profile and therapy, with native levels and directions. 50 items per page.

civicCuratedShowing 1–32 of 32 evidence items · levels, directions and significance as curated at the source; each row links to its CIViC record.

Therapy	Cancer	Type	Level	Direction · significance	Rating (1–5)	Status	Evidence	Source
DICER1 D1709E2
(predisposing)	Ovarian Sertoli-Leydig Cell TumorALIAS	Predisposing	C	Supports Predisposition	2	accepted

Cohorts

Alteration frequency by cohort (149)

Frequency = cases affected / cases profiled within one cohort. Cohorts are never pooled.

cbioportalObserved149 cohort rows (showing 50, sorted by frequency) · frequency = affected / profiled, as published by each cohort; cohorts are not pooled. Hover a row badge for its release.

Cohort	Mapped cancer	Alteration	Affected (n)	Profiled (n)	Frequency (%)	Rank in cohort	Source
Colorectal Cancer (MSK, Cancer Discovery 2022) coadread_mskresistance_2022	Colorectal Adenocarcinoma	Ssm

Literature

Linked publications (2)

25 per page, newest first.

pubmedPublishedShowing 1–2 of 2 publications · bibliographic data as recorded by PubMed; abstracts are not reproduced.

A survey of DICER1 hotspot mutations in ovarian and testicular sex cord-stromal tumors.
· Mod Pathol · 2015 · PMID 26428316 · validated civic_curation pubmed
Recurrent somatic DICER1 mutations in nonepithelial ovarian cancers.
· N Engl J Med · 2012 · PMID 22187960 · validated civic_curation pubmed

DICER1 D1709G2
(predisposing)	Ovarian Sertoli-Leydig Cell TumorALIAS	Predisposing	C	Supports Predisposition	3	accepted	EID5887 DICER1 mutations in the RNase IIIb domain were found in 29% of nonepithelial ovarian tumors (n=103) with high predominance in Sertoli-Lydig cell tumors (26/43; 60%). There was one mutation in D1709G. PMID 22187960 · Heravi-Moussavi et al., 2012 · Open in CIViC	civic
(oncogenic)	Granulosa Cell Tumor	Oncogenic	C	Supports Oncogenicity	1	accepted	EID5897 DICER1 mutations in the RNase IIIb domain were found in 29% of nonepithelial ovarian tumors (n=103) with one mutation in a Juvenile ovarian granulosa-cell tumor (1/14; 7%). This individual had a somat… (full text at CIViC) PMID 22187960 · Heravi-Moussavi et al., 2012 · Open in CIViC	civic

DICER1 D1709G2

(predisposing)

Ovarian Sertoli-Leydig Cell TumorALIAS

Predisposing

Supports Predisposition

accepted

EID5887

DICER1 mutations in the RNase IIIb domain were found in 29% of nonepithelial ovarian tumors (n=103) with high predominance in Sertoli-Lydig cell tumors (26/43; 60%). There was one mutation in D1709G.

PMID 22187960 · Heravi-Moussavi et al., 2012 · Open in CIViC

DICER1 D1709N3
(diagnostic)	Ovarian Sertoli-Leydig Cell TumorALIAS	Diagnostic	B	Does Not Support Positive	4	accepted	EID5534 In this exploratory study of Sertoli-Leydig cell tumors, DICER1 mutations were found in over half of the samples sequenced, but were not found to have any role in tumorigenesis, contrary to what was p… (full text at CIViC) PMID 26428316 · Conlon et al., 2015 · Open in CIViC	civic
(predisposing)	Ovarian Sertoli-Leydig Cell TumorALIAS	Predisposing	B	Supports Predisposition	3	accepted	EID5886 DICER1 mutations in the RNase IIIb domain were found in 29% of nonepithelial ovarian tumors (n=103) with high predominance in Sertoli-Lydig cell tumors (26/43; 60%). There were ten mutations in D1709N… (full text at CIViC) PMID 22187960 · Heravi-Moussavi et al., 2012 · Open in CIViC	civic
(oncogenic)	Glandular Pattern Ovarian Yolk Sac TumorUNRESOLVED	Oncogenic	C	Supports Oncogenicity	1	accepted	EID5899 DICER1 mutations in the RNase IIIb domain were found in 29% of nonepithelial ovarian tumors (n=103). There was one Yolk sac tumor that had a somatic mutation in D1709N. PMID 22187960 · Heravi-Moussavi et al., 2012 · Open in CIViC	civic

DICER1 Loss-of-function2
(predisposing)	Ovarian GynandroblastomaALIAS	Predisposing	A	Supports Predisposition	2	submitted	EID11479 In an analysis of 107 patients enrolled in the International Ovarian and Testicular Stromal Tumor Registry, 5 had gynandroblastoma (14-32 yo). 4 of the 4 gynandroblastoms with material available for … (full text at CIViC) PMID 29037807 · Schultz et al., 2017 · Open in CIViC	civic
〃	Anaplastic Sarcoma of the Kidney	Predisposing	B	Supports Predisposition	2	submitted	EID11473 Analysis of DICER1 mutations in tumor/normal pairs from pediatric patients (7 months to 13 yo) with anaplastic sarcoma of the kidney identified a somatic RNase IIIb DICER1 mutation in 8 of 9 cases (89… (full text at CIViC) PMID 28862265 · Wu et al., 2018 · Open in CIViC	civic