Juvenile Granulosa Cell Tumor of the Testis: A Case Presenting as a Small Round Cell Tumor of Childhood

Abstract

We describe the case of a testicular juvenile granulosa cell tumor (JGCT) in a 4-year-old boy. The highly undifferentiated appearance and robust mitotic activity of the neoplasm led to an initial impression of an aggressive, small round cell tumor of childhood. Immunocytochemical and ultrastructural studies excluded the usual members of that group, and led to the correct diagnosis. To our knowledge, this is the oldest reported patient to present with this tumor in the testis, and the first with clinical evidence of hormonal activity. The benign behavior of testicular JGCT mandates that it be distinguished from other, much more aggressive, neoplasms which it may resemble.

Juvenile Granulosa Cell Tumor of Testis: Case Report and Review of Literature

“…2,15,17 Although presence of hormonal activity was presumed to exclude JGCT, 7,14 one case has been reported showing hormonal activity. 9 As per retrospective analysis, JGCT can be associated with chromosomal abnormalities like 45X/XY mosaicism, complete XXY triploidy, and trisomy 12. 6,15,16 Our case has a normal 46XY karyotype, which was observed in the majority of the cases (35 of 43).…”

Congenital Intra-Abdominal Bilateral Juvenile Granulosa Cell Tumors of the Testis Associated with Constitutional Loss of Material from Chromosome 4

“…Juvenile granulosa cell tumor of the testis most commonly presents as a scrotal mass. Other reported presentations have included testicular torsion or gynecomastia [3,4]. It can occur in an undescended testis, either in the abdomen or in the inguinal canal [2,3].…”