PURPOSE:

To report the incidence, characteristics, treatment, and outcomes of bifocal germinomas treated with chemotherapy followed by focal radiation.

METHODS AND MATERIALS:

This was a retrospective review. Inclusion criteria included radiologic diagnosis of bifocal germinoma involving the pineal and neurohypophyseal region, no evidence of dissemination on spinal MRI, negative results from cerebrospinal fluid cytologic evaluation, and negative tumor markers.

RESULTS:

Between 1995 and 2004, 6 patients (5 male, 1 female; median age, 12.8 years) fulfilled the inclusion criteria. All had symptoms of diabetes insipidus at presentation. On MRI, 4 patients had a pineal and suprasellar mass, and 2 had a pineal mass associated with abnormal neurohypophyseal enhancement. All patients received chemotherapy followed by limited-field radiation and achieved complete remission after chemotherapy. The radiation field involved the whole ventricular system (range, 2,400-4,000 cGy) with or without a boost to the primary lesions. All patients remain in complete remission at a median follow-up of 48.1 months (range, 9-73.4 months).

CONCLUSIONS:

This experience suggests that bifocal germinoma can be considered a locoregional rather than a metastatic disease. Chemotherapy and focal radiotherapy might be sufficient to provide excellent outcomes. Staging refinement with new diagnostic tools will likely increase the incidence of the entity.