Clinicopathologic study of juvenile granulosa cell tumor of ovary

Objective To study the clinicopathologic features, diagnostic criteria and prognosticparameters of juvenile granulosa cell tumor of ovary. Methods The clinical and pathologic findings of 7cases of juvenile granulosa cell tumor were retrospectively reviewed. Immunohistochemical study was carriedout in 6 of these cases. The follow-up data were also analyzed. Results The mean age of the patients was24 years ( range = 6 to 53 years). Four patients presented with hormonal disturbance, while 3 patientspresented with abdominal pain or swelling. Six patients underwent unilateral salpingo-oophorectomy. Sixcases were in stage Ⅰ A and the remaining case in stage Ⅰ C. Follow-up information was available in 6patients and the duration of follow up ranged from 1 to 10 years (mean =4.3 years). Five patients remainedhealthy, with no evidence of tumor recurrence. One patient died of tumor metastasis one year after thediagnosis. Gross examination showed that the tumor masses ranged from 7 to 20 cm in the greatest dimension(average = 13.4 cm). Four of the 7 tumors were mixed solid-cystic in appearance and 2 cases wereunilocular cystic in nature. Microscopic examination showed diffuse atypical follicular structures formed bygranulosa cells. The granulosa cells contained round hyperchromatic nuclei, without nuclear grooves or Call-Exner body formation (6/7). In one of the cases studied, minor foci resembling adult granulosa cell tumorwere also demonstrated. The degree of cellular atypia varied (3 cases with severe atypia, 1 case withmoderate atypia and 3 cases with mild atypia). The mitotic count ranged from 1 to more than 5 per 10 highpower fields. Immunohistochemical study showed diffuse positivity for vimentin (6/6). The staining forcytokeratin ( AE1/AE3) and calretinin was negative. Four cases expressed CD99 and 1 case was positive forinhibin. Conclusions Juvenile granulosa cell tumor is characterized by the presence of diffuse atypicalfollicular structures formed by small round cells, without nuclear grooves or Call-Exner bodies. Rare casescontain minor foci of adult granulosa cell tumor. They can be unilocular cystic in nature. The degree of nuclear atypia, mitotic activity and size of the tumor vary and do not correlate with the risk of recurrence and aggressive biologic behavior. Key words: Ovarian neoplasms; Granulosa cell tumor; Diagnosis, differential; Immunophenotyping; Prognosis