Common Neoplasms of DICER1 Syndrome in Pediatric Pathology

Authors: Yamin Ma*

DOI: 10.23880/cprj-16000212

Volume 8, Issue 1

Abstract

DICER1 syndrome is an autosomal dominant tumor predisposition disorder with a DICER1 germline mutation, and it frequently appears in the practice of pediatric pathology. Here the common diseases of DICER1 syndrome have been reviewed with epidemiologic and histopathologic features, such as pleuropulmonary blastoma (PPB), cystic nephroma, embryonal rhabdomyosarcoma, and Sertoli-Leydig cell tumor. During routine practice, these histopathologic findings should be paid attention for the possible DICER1-related neoplasm and potential detection of DICER1 mutation clinically.

Keywords: DICER1; Pleuropulmonary Blastoma (PPB); Cystic Nephroma, Embryonal Rhabdomyosarcoma; Sertoli-Leydig Cell Tumor

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