Tumors of the Pineal Region

Pineal region tumors are rare, accounting for only 0.9% of all malignant CNS tumors. They are highly heterogeneous in presentation, histopathology, treatment, and prognosis, necessitating management in specialized centers. Due to their rarity, unpredictable behavior, complex surgical access, and potential neurological consequences, no standardized care guidelines exist. Brain and spine contrast-enhanced MRI, spectroscopy, PET imaging, and specific markers are essential to characterize the tumor and plan treatment. MRI of the brain and spine, along with cerebrospinal fluid (CSF) and blood tests, are crucial for staging and detecting tumor dissemination. Surgical decisions include biopsy (endoscopic, stereotactic) or open microsurgical procedures. In cases where serum and CSF markers are inconclusive, biopsy is recommended, although unclear histological diagnoses do not reduce survival rates. Maximal resection is unnecessary in germinomas, but for pineal parenchymal tumors (PPTs), gross total resection correlates with better survival outcomes. Stereotactic radiosurgery (SRS) is a valid alternative treatment, especially when combined with surgery or chemotherapy, but its efficacy depends on tumor histology. Although SRS without biopsy is performed in many cases, it remains controversial, as imaging advancements allow differentiation between germ cell and non-germ cell tumors. For germinomas, platinum-based chemotherapy helps reduce complications from radiation, lowering doses from 50 Gy to 24 Gy. Since overall survival is not impacted by whether patients have a definitive or unclear histological diagnosis, we believe that in selected cases, SRS without histological confirmation is a viable option. However, further studies are needed to validate this strategy.