Papillary Tumor of the Pineal Region in Children: Presentation of a Case and Comprehensive Literature Review

Background Papillary tumor of the pineal region (PTPR) is a rare grade II-III pineal lesion with peculiar histological and immunohistochemical features. These tumors mostly occur in adults, only rarely in children, with 19 cases reported up to now. Case Description We present a 3-year-old boy who underwent reoperation for a recurrent PTPR (grade II). Gross total resection of the lesion through an occipital interhemispheric approach with the patient in a sitting position was followed by adjuvant radiotherapy and chemotherapy. Histological examination revealed tumor progression (grade III) and an MIB-1 proliferation index >25%. The patient continues to do well with no evidence of recurrence more than 3 years following surgery. A comprehensive literature review regarding the PTPR, including the current management in children, is reported. Conclusions PTPRs are extremely rare in children, and immunohistochemistry is needed to differentiate them from other pineal tumors. These tumors show a high rate of recurrence, and a multidisciplinary management approach (microsurgical resection followed by radiotherapy and/or chemotherapy) can help achieve a favorable outcome.