Congenital Intra-abdominal Bilateral Juvenile Granulosa Cell Tumors of the Testis Associated with Constitutional Loss of Material from Chromosome 4
ABSTRACT Juvenile granulosa cell tumor (JGCT) is an uncommon gonadal stromal tumor that occurs rarely in the testis. We report a newborn boy with bilateral intra-abdominal JGCT presenting with abdominal distention and respiratory distress at birth. He was taken to the operating room emergently, and 2 large masses connected by gubernacula to the inguinal canals were resected. Associated abnormalities included a constitutional chromosome 4 abnormality, polymicrogyria, and renal cysts. This report describes a rare presentation of JGCT with abdominal compression and expands the literature to include bilateral testicular involvement. Additionally, it is the 1st report of JGCT associated with a chromosome 4 abnormality, highlighting a genetic region that may be important in JGCT development.
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ABSTRACT: Juvenile granulosa cell tumour (JGCT) of the testis is a benign neoplasm rarely seen in children. It usually presents as a unilateral scrotal mass and can be associated with genital ambiguity and chromosomal anomalies. Radical orchiectomy is the treatment of choice. We present an infant with a JGCT of the testis and we review the typical findings of the disease.Canadian Urological Association journal = Journal de l'Association des urologues du Canada 04/2012; 6(2):E101-3. DOI:10.5489/cuaj.10176 · 1.92 Impact Factor