Sertoli-Leydig Cell Tumour and DICER1 Mutation: A Case Report and Review of the Literature

Sertoli-Leydig cell tumours of the ovary (SLCT) are rare tumours predominantly caused by mutations in the DICER1 gene. We present a patient with a unilateral SLCT who had an underlying germline DICER1 gene mutation. We discuss the underlying pathology, risks, and screening opportunities available to...

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Bibliographic Details
Main Authors: B. Wormald, S. Elorbany, H. Hanson, J. W. Williams, S. Heenan, D. P. J. Barton
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Obstetrics and Gynecology
Online Access:http://dx.doi.org/10.1155/2018/7927362
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Summary:Sertoli-Leydig cell tumours of the ovary (SLCT) are rare tumours predominantly caused by mutations in the DICER1 gene. We present a patient with a unilateral SLCT who had an underlying germline DICER1 gene mutation. We discuss the underlying pathology, risks, and screening opportunities available to those with a mutation in this gene as SLCT is only one of a multitude of other tumours encompassing DICER1 syndrome. The condition is inherited in an autosomal dominant fashion. As such, genetic counselling is a key component of the management of women with SLCT.
ISSN:2090-6684
2090-6692