Abstract

Background: The aim of this study is to report clinical features and treatment outcome of pineal parenchymal tumors (PPTs). Methods: Between 2006-2017, medical records of 34 patients of PPTs were analyzed to study patterns of care of patients who received adjuvant radiotherapy (RT) with or without chemotherapy (CT) following surgery. Overall survival (OS) and progression-free survival (PFS) were analyzed by Kaplan- Meier method. Results: According to WHO classification, the study comprises of 19 pineoblastomas (PB), 11 pineal parenchymal tumors of intermediate differentiation (PPTID) and 4 pineocytomas (PC). Median age at presentation was 10, 26 and 30 years, respectively. Headache was commonest symptom (77%) followed by visual disturbance. 59% underwent surgical resection and 41% had biopsy only. Median MIB labelling index of PPTIDs was 6%, whereas it was 35% in PB and 2% in PC. Spinal drop metastasis was present in 8 patients with PB and 3 patients with PPTIDs. All PBs and PPTIDs with spinal drop metastasis (2) received cranio-spinal irradiation (CSI) and adjuvant CT. The rest of the PPTIDs and PC received focal RT to the brain. The commonest regimen was a combination of carboplatin and etoposide (CE), used in 6 (32 %) PB. CE with vincristine and IT-MTX was given in patients who developed drop metastasis subsequently. At a median follow-up of 16 months, 9 (47%) patients of PB and 3 (27%) of PPTID developed recurrence in cranio-spinal axis. 5 year progression-free survival was 41%, 62% and 75%, respectively. Median overall survival (OS) was 27, 50, 18 months, and 5 year OS was 38%, 47% and 75%, respectively. Conclusions: PPTs reflect a broad spectrum of malignant potential and prognosis. PB is an aggressive tumor and requires adjuvant CSI and CT following surgery. PPTIDs are relatively rare and require meticulous pretreatment evaluation of neuraxis and knowledge of pathological factors for management. Multi-institutional cooperation in the form of prospective studies is recommended in view of rarity of tumor. Legal entity responsible for the study: AIIMS, New Delhi. Funding: Has not received any funding. Disclosure: All authors have declared no conflicts of interest.

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