Radiotherapy for papillary tumor of the pineal region: A systematic review of the literature

Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial brain tumor, characterized by a high risk of local recurrence (greater than 70 % at 6 years). The aim of our study was to review the available literature on radiotherapy for PTPR in order to evaluate timings, schedules, outcomes and toxicities of this treatment modality. In our review, 72.4 % (84) of the patients diagnosed with PTPR received radiation therapy. There is heterogeneity in the dose prescription, ranging from 45 Gy (25 x 1.8 Gy) to 60 Gy (30 x 2 Gy) for 3D Conformal Radiation Therapy and from 12 Gy to 36 Gy for Stereotactic Radiosurgery. Being considered as a grade II or III tumor, PTPR should receive higher total radiation dose in the adjuvant setting. Our analysis showed a very limited treatment-related toxicity with an expected 10-y OS of 72.5 %. At 5-years from the diagnosis, about 60 % of the patients experienced a local recurrence, whereas at 10 years the rate is higher than 80 %. In the literature, conflicting data about radiotherapy for PTPR are reported, in particular regarding disease progression. Although radiotherapy represents a fundamental treatment in the management of PTPR, prospective studies are required to better define its impact on overall survival and progression-free survival.