BACKGROUND

Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial tumor with Central Nervous System (CNS) World Health Organization (WHO) grade II or III classification. Due to its rarity, there is no clear census on treatment. The purpose of this study is to identify the optimal treatment plan focused on extending overall survival (OS).

METHODS

This is an institutional case series with review of the literature. Fifty-three publications were analyzed. Only cases with histological diagnosis of PTPR were included. Data collected included demographics, treatment modalities, disease progression, and OS.

RESULTS

The analysis included 105 patients from the literature and 3 new cases (54 female, 50%) with an average age of 33.1 years (range 1-73 years). The average lesion size was 26.4 mm (range 5-50 mm) in longest axis. All patients underwent an initial resection. There were 46 cases of surgery alone. The remaining cases received adjuvant therapy including radiation (RT), stereotactic radiosurgery (SRS), chemotherapy (CT), or RT and CT. The average follow-up was 61.4 months (range 1-240 months). OS at 1 year was 96.9%, at 5 years was 87.5%, and at 10 years was 80.2%. Overall progression-free survival (PFS) was 57.4%. Statistical significance was observed in PFS in the surgery plus SRS group and surgery plus CT and RT group. Surgery with SRS had the best PFS (75%), and OS at 1 year (100%) and 5 years (88.9%). Surgery with CT and RT had the best OS at 10 years (85.7%).

CONCLUSION

We describe a case series and literature review of PTPR to help guide the most effective treatment strategies for this rare disease entity. We recommend surgery followed by SRS as the treatment of choice because of its best PFS and 5-year survival rates. We would also recommend adding chemotherapy in the event of disease progression or recurrence as adjuvant radiation and chemotherapy provided the best 10-year survival.