PTPS-27NON-GERMINOMATOUS GERM CELL TUMORS WITH SYNCHRONOUS PINEAL AND SUPRASELLAR INVOLVEMENT

Abstract

PURPOSE: Bifocal presentation of central nervous system (CNS) germinomas, with synchronous pineal and suprasellar involvement, is common, and associated with favorable outcome. However, the incidence and outcome of bifocal presentation of non-germinomatous germ cell tumors (NGGCT) is less well described. METHODS: Medical records of patients with CNS NGGCT treated at Children's Hospital Los Angeles between 1993 and 2014 were reviewed for clinical characteristics, treatment and outcome. RESULTS: Seven (24.1%) of 29 patients diagnosed with CNS NGGCT between 1993 and 2014 had bifocal involvement. The mean age at diagnosis was 13.1 years (range: 8-22 years). Diagnosis was made on tumor marker elevation in seven patients, and confirmed on histopathology on one patient. Seven patients had elevated AFP and one patient had elevated βHCG in serum and/or CSF. Two patients are still undergoing therapy and have not been included in the survival analyses. Four of remaining five patients are long term survivors at 41, 56, 57, and 97 months. Patient #1 received initial chemotherapy without irradiation, relapsed in the 3rd ventricle at 10 months, and was successfully salvaged with high-dose chemotherapy (HDCx) with autologous hematopoietic stem cell rescue (AuHCR) and 3600cGy craniospinal irradiation (CSI) with local boost. Patient #2 had partial response to chemotherapy, underwent HDCx and AuHCR, followed by 3600cGy CSI with boost. Patients #3 and #4 received chemotherapy with a complete response, followed by whole ventricular irradiation with a local boost. Patient #5 received chemotherapy only, developed ventricular relapse at 75 months, was treated with cyclophosphamide and vinblastine followed by CSI with boost, but died at 87 months. CONCLUSIONS: Our experience suggests that bifocal NGGCT may be more common than previously thought, and may be more likely to have yolk-sac component given elevated AFP, and therefore have a better prognosis than other NGGCT.