Radiation therapy of pineal region tumors: 25 new cases and a review of 208 previously reported cases

Abstract

Purpose: Malignant pineal region tumors are rare neoplasms arising in midline structures of the brain. This report analyzes the influence of histology, tumor location, radiation dose, treatment volume, age and cerebrospinal fluid findings on freedom from relapse, freedom from spinal relapse and survival.

Methods and materials: Patient and treatment parameters of 25 cases of pineal region tumors managed at Stanford University are presented, and an additional 208 published cases were reviewed. Univariate and multivariate analysis were performed to delineate parameters predictive of freedom from relapse, freedom from spinal relapse, and survival for all 233 patients.

Results: The 5- and 10-year freedom from relapse for Stanford patients was 63% and 46%, respectively. The 5- and 10-year survival for Stanford patients was 67% and 61%, respectively. The 5- and 10-year freedom from relapse for the total 233 cases was 66% and 61%, respectively. The 5- and 10-year survival for all patients was 74% and 68%, respectively. For the entire group, biopsy confirmed germinoma and non-biopsied tumors had superior freedom from relapse compared to non-germinoma germ cell tumors (p = 0.03, p = 0.005, respectively). Non-biopsied patients had improved survival compared to non-germinoma germ cell tumors (p = 0.004). Pineal parenchymal tumors had worse freedom from relapse compared to non-biopsied patients (p = 0.04). For patients with suprasellar tumors, germinomas were associated with improved freedom from relapse compared to non-germinoma germ cell tumors (p = 0.02). Simultaneous pineal and suprasellar tumors had superior survival compared to solitary tumors of pineal (p = 0.04), suprasellar (p = 0.03), or third ventricle location (p = 0.03). Twenty-two patients (9.4%) developed isolated spinal relapse. Five- and 10-year spinal relapse rates for all patients were 11% and 13%. Survival after spinal relapse was 19%. Pineal parenchymal tumors had lower freedom from spinal relapse compared to non-biopsied patients (p = 0.001). For tumors located in the pineal gland, germinomas and pineal parenchymal tumors had lower freedom from spinal relapse than did non-biopsied patients (p = 0.006, p = 0.004, respectively). Pineal germinomas had lower freedom from spinal relapse than germinomas with suprasellar location (p = 0.04). Univariate and multivariate analysis identified tumor histology as the most significant predictor of freedom from relapse, freedom from spinal relapse and survival.

Conclusion: Histologic type had the greatest impact on outcome. Treatment recommendations should be based on assessment of histologic type and extent of disease.