Intratubular Large Cell Hyalinizing Sertoli Cell Tumor of the Testes in a 4-Year-Old Male With Peutz-Jeghers Syndrome.

Document Type

Article

Publication Date

4-1-2015

Abstract

Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder that typically displays familial inheritance. Gastrointestinal polyposis and cutaneous pigmentation is a classic presentation of this syndrome. The reported lifetime cumulative cancer risk in PJS patients is >76% when compared with the general public with females affected more often than males. The prepubertal testicular tumor registry found Sertoli cell tumors (SCTs) to compose approximately 1% of all pediatric solid tumors. Prepubertal testicular masses are relatively rare. Only a small number of SCT cases have been reported in the first decade of life. The concurrence of PJS and feminizing SCTs of the testes is an increasingly recognized cause of prepubertal gynecomastia. The testicular lesions observed in patients with PJS primarily represent multifocal intratubular large cell hyalinizing SCTs with a distinct morphology that differs from large cell calcifying SCTs and sex cord tumors with annular tubules. Here, we describe the diagnosis and treatment course of a 4-year-old male with a SCT of the testes and diagnosis of PJS.

Publication Title

Journal of pediatric hematology/oncology : official journal of the American Society of Pediatric Hematology/Oncology

ISSN

1536-3678

Volume

37

Issue

3

First Page

184

Last Page

187

DOI

10.1097/MPH.0000000000000243

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