Metastatic granulosa cell tumor with pattern of sex cord tumor with annular tubules.

A case of malignant, complex sex cord tumor with multiple differentiations in a 38-year-old woman is presented. The neoplasm presented originally as a pure granulosa cell tumor, and in the second look operation as a sex cord tumor with annular tubules (SCTAT). Six years later abdominal metastases revealed a mixed pattern of granulosa and Sertoli cell tumor admixed with SCTAT. The patient died despite chemotherapy. Analysis of the 10 previously reported cases of malignant SCTAT shows that tumor size is the main prognostic indicator. Mitoses, atypia, and stromal invasion seem also to bear prognostic implications when present. The association of both granulosa and Sertoli cells with SCTAT seems to provide a clue for SCTAT being a peculiar intermediate phenotypical expression of pluripotential stem sex cord cells of the gonads.