Germ Cell Tumors (GCTs) are 2% of intracranial neoplasms, mainly in the pineal/suprasellar region and in young ages. The overall prognosis, in tumors containing a non-germinomatous component, is poor with a median 5 year overall survival (OS) of under 30%. Treatment recommendations suggest a multimodal approach
We performed a retrospective review of all consecutive primary intracranial GCT patients (pts) diagnosed and treated at our Institution from 1988 to 2015. Primary aim: to characterize the clinical, demographic and treatment data. Secondary aim: to evaluate overall survival (OS) at 5 and10 years using the Kaplan-Meier method and related prognostic factors
From a total of 45 pts, 30 were males, median age 11 years (P10-90: 5,75–20). The main symptoms were cephalalgia (45%), diabetes insipidus (31%) and vomiting (20%). 53% had endocrinologic disturbances, 44% visual field limitations and 20% pts Parinaud Syndrome. Sixty percent presented with intracranial hypertension. Primary location was the pineal and suprasellar in 56% and 29% of cases. Cranial and Neuroaxial Magnetic Ressonance Imaging (MRI) was the preferred imaging method used in 91 and 53% pts, respectively. The diagnosis was reached by tumour markers in 22,2%, tumour biopsy in 26,6% and surgery in 51,1% pts. Tumour markers were elevated in 69% pts. Fourty-ninve percent of pts had pure germinoma, 15,5% pts had mixed germinoma and 31% pts non-germinoma. Sixty-nine percent of pts underwent intracranial decompression techniques. Sixty-nine percent of pts had chemotherapy regimens (PEI in 18 pts) and 82,2% pts had cranial radiotherapy (with simultaneous neuroaxial irradiation in 17 pts). Complete response was achieved in 91,1% of pts with 22,2% pts recurring. The 5 and 10 OS rate was 88 and 85% respectively (98 and 98% for Germinomas and 82 and 75% for non-germinoma). OS values differences between histologies did not reach statistical significance. In the multivariate analysis only cranial radiotherapy and absence of recurrence were associated with improved survival (p = 0.003 and p = 0.016, respectively).
First line multimodality treatment achieves good clinical outcomes, with focus on cranial radiotherapy. Disease recurrence is associated with worse outcomes.
Clinical trial identification
Legal entity responsible for the study
Instituto Portugues de Oncologia de Lisboa
J.P. Silva: Travel grants to Oncology congresses by Bristol-Myers Squibb. All other authors have declared no conflicts of interest.