Faculty Collaboration Database

Pediatric sex cord-stromal tumor with composite morphology: a case report. Pediatr Dev Pathol 2005;8(6):680-4

Date

10/14/2005

Pubmed ID

16222477

DOI

10.1007/s10024-005-0055-2

Scopus ID

2-s2.0-29244454271 (requires institutional sign-in at Scopus site)   7 Citations

Abstract

A 12-year-old female with developmental delay/mental retardation and a family history of gynecologic cancers presented with nonspecific abdominal complaints and was found to have a 4.5-kg, 25- x 23- x 15-cm pelvic mass with solid and cystic components and associated retroperitoneal and mesenteric lymphadenopathy. Laboratory studies revealed increased serum levels of CA-125 and inhibin B. Histologically, the tumor exhibited several different morphologic appearances including adult granulosa cell tumor, juvenile granulosa cell tumor (with areas of marked atypia), and Sertoli cell tumor. Immunohistochemically, the tumor was positive for calretinin, MIC-2 (CD99), S100 protein, PGP 9.5, and neuron-specific enolase. Electron microscopy of the Sertoli cell tumor-like areas showed Charcot-Bottcher filaments, a distinguishing feature of Sertoli cells. Together, these findings supported a diagnosis of mixed sex cord-stromal tumor including granulosa cell tumor of adult and juvenile types and intermediate- to high-grade Sertoli cell tumor, with large areas of markedly atypical sex cord-stromal tumor.

Author List

Jarzembowski JA, Lieberman RW

Author

Jason A. Jarzembowski MD, PhD Sr Associate Dean, CEO CSG, Professor in the Pathology department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Carcinoma, Small Cell
Child
Developmental Disabilities
Diagnosis, Differential
Female
Humans
Immunohistochemistry
Intellectual Disability
Lymphoma
Microscopy, Electron, Transmission
Neuroectodermal Tumors, Primitive
Ovarian Neoplasms
Rhabdomyosarcoma
Sarcoma, Ewing
Sex Cord-Gonadal Stromal Tumors