Papillary Tumor of the Pineal Region with Leptomeningeal Seeding: A Case Report and Literature Review

Papillary tumor of the pineal region (PTPR) is an infrequent neoplasm arising from the ependymal cells of the sub-commissural organ. This tumor entity was incorporated into the World Health Organization (WHO) classification of central nervous system tumors in 2007. Given the propensity for local recurrence observed in PTPR cases and the documented instances of leptomeningeal seeding in previous case reports, it presents a substantial risk of significant morbidity. Due to its rarity, there is no established standard for its management. Surgical intervention constitutes the primary treatment modality, while the role of adjuvant radiotherapy remains ambiguous. In this case report, we present the clinical course of a 46-year-old male diagnosed with PTPR who underwent surgical resection followed by adjuvant radiotherapy. 14 months post-initial treatment, the patient manifested intracranial and spinal metastases in the form of leptomeningeal dissemination. Subsequently, systemic chemotherapy utilizing vincristine and carboplatin was initiated, and the patient exhibited no evidence of disease progression over the last six months.