Gynandroblastoma with elements resembling juvenile granulosa cell tumor

Gynandroblastoma is an extremely rare sex cord-stromal tumor that exhibits significant ovarian and testicular differentiation. In most previously reported tumors, adult granulosa cell tumor has formed the ovarian-type component and Sertoli or Sertoli-Leydig cell tumor (SLCT) has formed the testicular-type component. In contrast, the ovarian-type element in the case reported here resembled juvenile granulosa cell tumor (JGCT). The testicular-type elements accounted for 20% of the tumor and resembled intermediate-grade SLCT. The Sertoli cells had strong cytoplasmic staining for cytokeratin CAM 5.2 and positive nuclear staining with estrogen and progesterone receptor, whereas the JGCT-like areas were negative for these antibodies. Ultrastructurally, the JGCT-like areas consisted of groups of cells that were invested by a basal lamina and had low nuclear-cytoplasmic ratios, cytoplasmic lipid droplets, and simple junctional complexes. The Sertoli cells in the SLCT-like areas had long, tight junctions and well-formed desmosomes. Gynandroblastoma usually presents clinically as an abdominal mass, often associated with either virilizing or feminizing manifestations. The prognosis is favorable and similar to that of the individual tumor components, but clinical follow-up in the small number of cases has been limited.