Clinical experience and outcomes in patients with pineal parenchymal tumor of intermediate differentiation (PPTID): a single-institution analysis.

Abstract

Pineal parenchymal tumor of intermediate differentiation (PPTID) is a neoplasm of pinealocyte origin and of intermediate differentiation (WHO grade 2 or 3). Treatment selection and prognostication is challengingfor this rare, recently characterized tumor. In this single study, we review our clinical experience in patients with PPTID as well as pooled data from two other institutions. This is a retrospective analysis of patients seen at Mayo Clinic, with additional data pooled from two similar studies at outside institutions for comparison and further analysis. Ten adult patients (6 male) were identified. Median age at diagnosis was 36years (range 13-73years). Patients were followed between 3 and 88months with no reported deaths. The most common presenting symptom was headache, followed by nausea. Nine patients had local disease without neuroaxial dissemination at diagnosis, one of which had tumor recurrence at 36months. Two patients underwent gross total resection (GTR) without adjuvant radiation without recurrence (PFS 4 and 46months). In our combined analysis, we reviewed 36 patients from three different institutions. Four patients presented with disseminated disease. GTR was achieved for 16 patients. Twenty-eight patients received adjuvant radiation therapy and 16 patients experienced disease recurrence. Median overall survival was 44months. Our single institution experience and combined multi-institution analysis suggest GTR is associated with improved outcomes. The role of adjuvant radiation therapy and utility of CSI compared to focal RT is less unclear. Prospective study is required to identify optimal adjuvant therapy selection.