Pediatric Pathology

ISSN: 0277-0938 (Print) (Online) Journal homepage: http://www.tandfonline.com/loi/ipdp17

Juvenile Granulosa Cell Tumor F. Raafat, H. Klys & G. Rylance To cite this article: F. Raafat, H. Klys & G. Rylance (1990) Juvenile Granulosa Cell Tumor, Pediatric Pathology, 10:4, 617-623, DOI: 10.3109/15513819009067150 To link to this article: http://dx.doi.org/10.3109/15513819009067150

Published online: 09 Jul 2009.

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Date: 07 May 2016, At: 04:11

JUVENILE GRANULOSA CELL TUMOR

F. Raafat, MD, MRCPath, and H. Klys, BSc 0

Department of Pathology, Birmingham Children’s Hospital, Birmingham B 16 8ET, United Kingdom

G. Rylance, MB, MRCP

0 Department of General Pediatrics, Birmingham Children’s Hospital, Birmingham B16 8ET, United Kingdom

A 2-year-old girl presented with isosexual precacio, and a juvenile granulosa cell tumor of the left ovary was removed. The tumor was unusual in that striated muscle differentiation of the tumor cells was demonstrated. This suggests that the mesenchymal cells retain the potential for divergent differentiation.

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KEY WORDS: granulosa cell, ovaty, myoflaments, isosexual puberty

INTRODUCTION The granulosa cell tumor of the ovary is uncommon before puberty, and only 5% of these tumors occur in premenarchal girls (1). There are histological, immunohistochemical, and electron microscopic similarities between the juvenile granulosa cell tumor (JGCT) and its counterpart in adult women (13). Although there is abundant literature on the pathology of granulosa cell tumors of adults, there is less information on the morphologic findings of JGCT, which may reflect its relative rarity. We report a case of JGCT in a 2-year-old girl who had isosexual precocity and emphasize immunohistochemical and electron microscopic evidence of myogenic differentiation. This further supports the theory of their mesenchymal origin. Although the behavior of these tumors is unpredictable, the malignant variant recurs and metastasizes early in the course of the disease. The stage of the tumor at presentation remains the most reliable predictor of the outcome. The patient remains symptom-free 15 months after surgery. The authors wish to thank Mr. A. Brownhill for photography; Mrs. E. Halloran, Mrs. C . Evans, Mrs. R. Gellion, and Mr. M . Peck for immunohistochemistry and electron microscopy; Miss M. Aitken and Mrs. J. M. Clarke for secretarial assistance. Address reprint requests to Dr. F. Raafat, Department of Pathology, Children’s Hospital, Birmingham B16 8ET, United Kingdom.

Pediatric Patholou, 10:617-623, 1990 Copyrkht 0 1990 by Hemisphcre Publishing Corporation

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CASE REPORT A 2-year-old girl presented with signs of precocious puberty. Her mother noticed that she had developed fine pubic hairs and some breast enlargement during the previous 6 months. She had vaginal bleeding in the 2 weeks before presentation. There was palpable breast tissue bilaterally, a fair amount of dark pubic hair and signs of recent vaginal bleeding. A hard round mass approximately 10 cm in diameter was palpable in the midline of the lower abdomen. Complete general and detailed neurological examinations were normal. Investigation for precocious puberty showed normal values for folliclestimulating hormone (less than 1. O l u/L: reference range < 3 iu/L), luteinizing hormone (2.2 iu/L: range < 8 iu/L), and dehydroeprandrosterone sulphate (0.6 umol/L; range

Juvenile granulosa cell tumor.

A 2-year-old girl presented with isosexual precocity, and a juvenile granulosa cell tumor of the left ovary was removed. The tumor was unusual in that...
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