Pediatric sex cord-stromal tumor with composite morphology: a case report

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 ly...

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Published inPediatric and developmental pathology Vol. 8; no. 6; pp. 680 - 684
Main Authors Jarzembowski, Jason A, Lieberman, Richard W
Format Journal Article
LanguageEnglish
Published United States SAGE PUBLICATIONS, INC 01.11.2005
Subjects
Carcinoma, Small Cell - pathology
Child
Developmental Disabilities - complications
Diagnosis, Differential
Female
Humans
Immunohistochemistry
Intellectual Disability - complications
Lymphoma - pathology
Microscopy, Electron, Transmission
Neuroectodermal Tumors, Primitive - pathology
Ovarian Neoplasms - complications
Ovarian Neoplasms - metabolism
Ovarian Neoplasms - pathology
Rhabdomyosarcoma - pathology
Sarcoma, Ewing - pathology
Sex Cord-Gonadal Stromal Tumors - complications
Sex Cord-Gonadal Stromal Tumors - metabolism
Sex Cord-Gonadal Stromal Tumors - pathology
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Summary: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.
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ISSN:1093-5266
1615-5742
DOI:10.1007/s10024-005-0055-2