DICER1-associated central nervous system sarcoma in children: comprehensive clinicopathologic and genetic analysis of a newly described rare tumor.

doi:10.1038/s41379-020-0516-1

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Article

DICER1-associated central nervous system sarcoma in children: comprehensive clinicopathologic and genetic analysis of a newly described rare tumor.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc - 1 Oct 2020

Kamihara Junne, Paulson Vera, Breen Micheál A, Laetsch Theodore W, Rakheja Dinesh, Shulman David S, Schoettler Michelle L, Clinton Catherine M, Ward Abigail, Reidy Deirdre, Pinches R Seth, Weiser Daniel A, Mullen Elizabeth A, Schienda Jaclyn, Meyers Paul A, DuBois Steven G, Nowak Jonathan A, Foulkes William D, Schultz Kris Ann P, Janeway Katherine A, Vargas Sara O, Church Alanna J

Abstract excerpt

The spectrum of neoplasms associated with DICER1 variants continues to expand, with the recent addition of primary "DICER1-associated central nervous system sarcoma" (DCS). DCS is a high-grade malignancy predominantly affecting pediatric patients. Six pediatric DCS were identified through a combination of clinical diagnostic studies, archival inquiry, and interinstitutional collaboration. Clinical, histologic,...

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PMID: 32291395
DOI: 10.1038/s41379-020-0516-1

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