Two illustrative cases of papillary tumor of the pineal region: Clinical presentation, management, and outcomes.

Background: Papillary tumors of the pineal region (PTPR) are rare brain tumors thought to originate from specialized ependymal cell remnants. Their clinical behavior varies, typically manifesting as symptoms of increased intracranial pressure. Due to their rarity, literature on PTPR is limited, complicating the development of evidence-based treatment guidelines. We present two illustrative cases describing their diagnostic workup, management and respective outcomes.

Case description: This article presents two cases of PTPR, both diagnosed in adult females with a shared clinical presentation of hydrocephalus-associated symptoms. The first case, a 47-year-old patient, presented in October 2014 with progressive headaches, nausea, and vomiting. The second case, a 40-year-old patient, presented in October 2020 with a two-week history of diplopia, headache, and imbalance. Both patients underwent operative interventions, including biopsy and ventriculostomy, followed by tumor resection. Histological analysis confirmed PTPR in both cases, classified as WHO grade 2-3 and grade 3, respectively. The two cases also received radiation therapy, with case 1 demonstrating longstanding tumor control throughout follow-up, and case 2 experiencing aggressive metastatic activity in spite of repeated onco-surgical treatment.

Conclusion: These cases highlight the complex management of PTPR as well as the significance of tailored interventions and long-term surveillance for improved outcomes. Surgical resection, in conjunction with adjuvant or salvage high dose radiation, remains of essence.