Background: This study examined the outcome of patients with histologically confirmed pineal region tumors.
Methods: One hundred thirty-five patients with histologically confirmed pineal tumors and other germ cell tumors of the brain were evaluated retrospectively. The pineal parenchymal tumors (PPTs) included 15 pineoblastomas (PB), 2 mixed PPTs, 4 PPTs with intermediate differentiation, and 9 pineocytomas. The germ cell tumors included 48 germinomas, 26 mixed germ cell tumors, 11 mature teratomas, 9 immature teratomas, 6 malignant teratomas, 2 yolk sac tumors, and 3 choriocarcinomas. Patients were treated with various combinations of chemotherapy, radiotherapy, and surgery. The duration of follow-up ranged from 0.25 to 37.3 years, with a median follow-up of 5.3 years.
Results: The 5-year patient survival rate was 86% for those with mature teratomas; 86% with pineocytomas; 80% with germinomas; 67% with immature teratomas; 49% with PPTs, excluding pineocytomas; 38% with mixed germ cell tumors; and 17% with other germ cell histologies (P = 0.0001). The delivery of > 44 Gray (Gy) to germinomas and > 50 Gy to PPTs and nongerminomatous germ cell tumors (NGGCTs) other than mature and immature teratomas was associated with improved survival. A greater extent of resection was associated with a higher rate of survival in all patients with NGGCTs. The administration of chemotherapy was associated with improved survival in those patients with NGGCTs other than mature and immature teratomas.
Conclusions: Prognosis was dependent on tumor type. Obtaining a tissue diagnosis made it possible to tailor therapy according to tumor type and potentially improve the survival of patients. Survival was dependent on the dose of radiation administered to patients with PPTs, germinomas, and NGGCTs other than mature and immature teratomas. More extensive resection and the use of chemotherapy were also associated with improved survival in subgroups of patients with NGGCTs. Treatment recommendations are described in detail in the article.