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Article

DICER1 -Related Primitive Polyphenotypic Neoplasm : A Report of 15 Cases of an Underrecognized Tumor of the Gynecologic Tract and Peritoneum.

The American journal of surgical pathology - 1 May 2026

Devins Kyle M, Lin Lawrence Hsu, Fisch Adam S, Dias-Santagata Dora, Pinto Andre, Young Robert H, Oliva Esther, Turashvili Gulisa

Abstract excerpt

Somatic or germline pathogenic/likely pathogenic variants in DICER1 have known associations with certain neoplasms in the gynecologic tract, including Sertoli-Leydig cell tumors, embryonal rhabdomyosarcoma, and adenosarcoma. However, recent studies have highlighted DICER1- related malignant neoplasms with complex admixtures of sarcomatous, primitive glandular, and/or neuroectodermal elements, which are...

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Identifiers

PMID: 41801179
DOI: 10.1097/PAS.0000000000002517

Genes

DICER1
AFP
SALL4

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

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DICER1 -Related Primitive Polyphenotypic Neoplasm : A Report of 15 Cases of an Underrecognized Tumor of the Gynecologic Tract and Peritoneum.PMID 41801179Read ↗

DICER1-associated Tumors in the Female Genital Tract: Molecular Basis, Clinicopathologic Features, and Differential Diagnosis.Semantic proximity Read ↗

DICER1-sarcomas of GYN tract: Expanding on an emerging entity.Semantic proximity Read ↗

DICER1-associated embryonal rhabdomyosarcoma and adenosarcoma of the gynecologic tract: Pathology, molecular genetics, and indications for molecular testing.Semantic proximity Read ↗

The Histological Spectrum of DICER1-Associated Neoplasms.Semantic proximity Read ↗

Clinicians Practicing Obstetrics and Gynecology Are Uniquely Situated to Recognize DICER1 Syndrome.Semantic proximity Read ↗

Embryonal rhabdomyosarcoma of the uterine corpus: a clinicopathological and molecular analysis of 21 cases highlighting a frequent association with DICER1 mutations.Semantic proximity Read ↗

Pleuropulmonary blastoma-like peritoneal sarcoma: a newly described malignancy associated with biallelic DICER1 pathogenic variation.Semantic proximity Read ↗

DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.Semantic proximity Read ↗

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Estrogen Receptor Expression in DICER1-related Lesions is Associated With the Presence of Cystic Components.Semantic proximity Read ↗

Gynecologic Manifestations of the DICER1 Syndrome.Semantic proximity Read ↗

DICER1-related Sertoli-Leydig cell tumor and gynandroblastoma: Clinical and genetic findings from the International Ovarian and Testicular Stromal Tumor Registry.Semantic proximity Read ↗

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