Papillary tumor of the pineal region. Report of two cases and literature review

Papillary tumor of the pineal region (PTPR) was introduced to the WHO classification in 2007. This rare tumor of little known natural history and unpredictable behavior was described in fewer than 100 cases. Its optimal treatment is not established yet. We report another two cases of PTPR in whom tumors were totally removed via supracerebellar infratentorial approach and both were treated with radiotherapy. In a 37-year-old man the operation was delayed 6 years after the first tumor diagnosis and subsequent shunt placement. He has no complaints 10 years after the onset of the disease. A 45-year-old woman has no complaints 24 months after surgery. Our experience and the data from literature indicate that a total tumor removal is the major prognostic factor.