Parenchymal tumor of the pineal region: insights from a rare case and contemporary literature review

Abstract Background Pineal parenchymal tumors of intermediate differentiation (PPTIDs) are uncommon neoplasms representing a small subset of pineal region tumors. Their rarity, variable biological behavior, and evolving pathological classification make management challenging, particularly in the absence of standardized treatment guidelines. Case presentation A 63-year-old man presented with intermittent holocranial headache associated with vomiting. Imaging demonstrated a heterogeneously enhancing lobulated lesion in the pineal region. The patient underwent near-total tumor excision. Biopsy revealed a pineal parenchymal tumor of intermediate differentiation, CNS WHO grade 3. Following multidisciplinary tumor board discussion, focal radiotherapy was administered without chemotherapy and the patient achieved a favorable neurological recovery. Conclusions Management of PPTID requires a tailored strategy that balances disease control against treatment-related morbidity. This case demonstrates that near-total resection followed by focal radiotherapy may provide an effective treatment option for localized WHO grade 3 PPTID in selected patients, underscoring the importance of multidisciplinary decision-making in these rare tumors.