Juvenile granulosa cell tumor associated with rapid distant metastases

We have encountered a case of juvenile granulosa cell tumor, first described by SCULLY as a specific form of granulosa cell tumor, in a 23-year-old, nulliparous female associated with some unusual clinicopathological features. The tumor showed a diffuse or macrofollicular pattern with eosinophilic or clear, often vacuolated cytoplasm containing abundant lipid. Ultrastructures revealed features very similar to those of previously reported juvenile granulosa cell tumor. Intracytoplasmic filaments were observed, but smooth surfaced endoplasmic reticulums were not evident. The patient succumbed unexpectedly rapidly due to recurrence and distant metastasis, in spite of anti-cancer therapy. It is also interesting that surprisingly high levels of estrogens as well as testosterone in both urine and serum was recorded before surgery and after the development of recurrence.