Pineal Tumors and Pineal Region Tumors

Tumors of the pineal region (PRTs) are rare and heterogeneous, accounting for less than 1% of intracranial neoplasms. They encompass pineal parenchymal tumors (PPTs), germ cell tumors (GCTs), and other histologies, such as gliomas or meningiomas. Radiotherapy remains a cornerstone of their management, with techniques and doses tailored to tumor biology and dissemination risk.

This review summarizes current principles and practices in radiotherapy for PRTs, based on tumor subtype and extent of disease.

For pineocytoma (WHO grade I), gross total resection (GTR) provides excellent outcomes, while adjuvant radiotherapy (50-54 Gy) or stereotactic radiosurgery (SRS) is reserved for subtotally resected cases. Pineal parenchymal tumors of intermediate differentiation (PPTIDs) (grades II-III) display variable aggressiveness; GTR remains the main prognostic factor, and adjuvant RT (50-54 Gy) improves overall survival, with craniospinal irradiation (CSI, 23-36 Gy + boost) indicated in disseminated disease. Pineoblastoma (PB) is an aggressive embryonal tumor requiring multimodal therapy: CSI (36 Gy + boost to 54-55.8 Gy) combined with chemotherapy yields 5-year OS rates >70% in children ≥3 years, though outcomes remain poor in younger or metastatic patients. Papillary tumor of the pineal region (PTPR) (WHO II-III) typically recurs locally despite surgery; adjuvant focal radiotherapy (≈50 Gy) is recommended. The recently described desmoplastic myxoid tumor, SMARCB1-mutant shows intermediate behavior, with limited data supporting focal RT ≥54 Gy. Among germ cell tumors, pure germinomas-highly radiosensitive-achieve >90% 5-year OS with reduced-volume RT whole-ventricular irradiation (WVI, 24 Gy + boost) has replaced historical CSI. Non-germinomatous GCTs (NGGCTs) require combined chemotherapy and RT; CSI + boost achieves 70-90% 5-year OS. Proton therapy is increasingly employed to limit long-term neurocognitive and secondary cancer risks.

Management of pineal region tumors relies on an integrated approach combining surgery, radiotherapy, and, when indicated, chemotherapy. Advances in conformal and particle techniques have optimized the therapeutic ratio, while molecular classification now guides risk-adapted treatment. Future studies should refine dose-volume strategies and integrate biological markers to further personalize care.