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Testicular Juvenile Granulosa Cell Tumor - a Rare Entity that May Have Mildly Elevated Serum Alpha Fetal Protein in Infants

We present a rare case of testicular juvenile granulosa cell tumor (JGCT) in a 10-month-old male who had mildly elevated alpha-fetoprotein (AFP). The patient had a history of undescended right testis, and 2 cystic masses were identi ﬁ ed on the lower pole during orchidopexy planning. There was mild AFP elevation at 60 ng/mL (reference range: < 8.9 ng/mL) and LDH at 320 U/L (reference range: 125-220 U/L). The patient initially underwent piecemeal excision of the cystic masses. Microscopically, the largest cyst was lined by variable layers of granulosa cells, resembling a macrofollicle (Figure 1A). Within the cyst wall, multiple smaller ovarian follicle-like structures are present, comprising small clear to eosinophilic cells (Figure 1B). The tumor cells are immunopositive for inhibin and calretinin, while neg-ative for SALL4 (Figure 1C). These ﬁ ndings support a diagnosis of JGCT. Postoperatively, his AFP slowly trended up from 29 to 57 ng/mL over 6months, and LDH increased from 369 to 421 U/L. Ultrasound examination of the scrotum revealed a small cystic focus in the right testis. With up-trending AFP and a history of cryptorchism, a radical orchiectomy was performed. A .2-cm residual JGCT was seen at the edge of the prior surgical site (Figure 1D).