Papillary Tumors of Pineal Region: A Single-Center Experience in Management of 11 Cases

Abstract

BackgroundPapillary tumors of pineal region (PTPR) constitute a very rare subset of pineal region tumors that have been recently described. There is a scarcity of literature on the management and outcome of PTPR owing to the rarity of these tumors. To address this lacuna we analysed our experience in management of PTPR.Materials & Methods: In this study, we retrospectively analyzed the outcome of 11 patients, with histopathologically proven PTPR, who underwent surgical excision at our center. ResultsThe mean age was 33.3 years (range:12-45 years) and male:female ratio 1.75:1. Headache was the most common presentation followed by visual disturbances, altered sensorium, Perinaud’s syndrome and seizures. 6 patients required CSF diversion. Krause approach was the most common approach used for tumor excision (in 9 of 11 cases). There was no perioperative mortality. Two patients were lost to followup. In rest 9 patients, the average follow up period was 45 months (range: 12-79 months). On first postoperative MRI, 8 patients showed no evidence of residual tumor (GTR) while one patient had small residual tumor (NTR) that remained stable during followup. Four patients underwent adjuvant chemo-radiotherapy. None of the patients developed recurrence during followup. ConclusionPTPR is a rare subgroup of pineal region tumors with distinct cells of origin but presentation similar to other pineal region tumors. Surgical resection constitutes the mainstay of management and the extent of resection appears to be the most important determinant of prognosis. The role of adjuvant therapy, however, still needs to be determined.