Assessment of Optimal Treatment Plan for Papillary Tumor of the Pineal Region

Introduction: Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial tumor with CNS WHO grade II classification. Due to its rarity, there is no clear census on treatment following identification of this lesion. The purpose of this study is to identify the optimal treatment plan focused on extending overall survival for these patients.

Methods: This is an institutional case series with review of the literature limited by the extremely rare occurrence of PTPR. Forty-six publications were analyzed. Data collected included demographics, treatments (chemotherapy (CT), radiation (RT), stereotactic radiosurgery (SRS), and surgery (S)), and outcomes (survival and progression).

Results: 108 patients (54 female, 50%) were included in this study with an average age of 33.1 years (range 1-73 years). The average lesion size was 26.4 mm in longest axis. Of these patients, 8 cases had biopsy alone, 36 cases had S alone (26 gross total resections, 10 partial resections), 29 cases had S followed by RT, 6 cases had S followed by SRS, 2 cases had S followed by CT, 12 cases had S followed by RT and CT, and 15 cases had biopsy followed by RT without surgical resection. Average follow-up was 59.5 months. Overall survival at 1-year was 96.9%, 5-years was 85.4%, and 10-years was 71.7%. Overall disease progression of the cohort was 42.6%. Kaplan-Meier curves of the various treatment combinations revealed surgery with SRS offered the best overall survival of 100% at 5-years.

Conclusion : We describe a case series and literature review of PTPR to identify the most effective treatment for this rare disease entity. We report the treatment with the best overall survival. From our study, we recommend surgery followed by SRS as the treatment of choice providing the best survival rate for patients with PTPR.