Pineal Region Tumors

Tumors involving the pineal gland or body are uncommon. Insights into the molecular mechanisms of these tumors have led to new classifications. In the 2021 WHO classification of pineal region tumors, pineal parenchymal tumors include five distinct subtypes such as pineocytoma (PC), pineal parenchymal tumors of intermediate differentiation (PPTID), papillary tumor of the pineal region (PTPR), pineoblastoma (PB), and desmoplastic myxoid tumor of the pineal region, SMARCB1-mutant. Neuroradiological imaging plays a key role in the diagnosis, surgical planning, and follow-up of patients with pineal masses. The mainstay of treatment for pineal gland tumors is surgery. Radiation therapy remains an essential part of multidisciplinary care with its use dependent upon the histologic subtype, grading, extent of disease, and combination with chemotherapy. In general, adjuvant treatment is indicated for subtotal resections and high-grade tumors. Craniospinal irradiation is indicated for tumors with the potential for leptomeningeal and spinal dissemination.