RARE-20. Retrospective analysis of 9 pineoblastoma

BACKGROUND: Pineoblastomas (PBs) are rare, supratentorial, primitive neuroectodermal tumors. Little is known with the clinical features and outcomes of PBs. METHODS: We retrospectively analyzed consecutive patients with PBs who were treated in Guangdong Sanjiu Brain Hospital between December 2006 to May 2020. RESULTS: A total of 9 patients (7 males and 2 females) with PBs were treated in our hospital with a median age of 9 yrs (range: 1-36 yrs) at diagnosis. Total or near-total resection was achieved in 3 patients (33%), partially resection in 4 (44.4%),and biopsy in 2(22.2%). There were 4 patients have spinal cord metastasis at diagnosis. Five patients received craniospinal irradiation (CSI), with concurrent or adjuvant chemotherapy. The average total dose of CSI was 34.80±2.683Gy, and the average dose to local tumor bed was 56.08±6.41Gy. Two patients younger than 3 years old only received chemotherapy, while 1 patient did not receive any postoperative treatment, and 1 patients was unknown. The median followed up time is ? months(range:3-39 months). At the last follow up, 5 patients were died, 3 patients were survived, and 1 was lost to follow-up. The median OS was 31 months (95%CI 1.782-60.281). Disease progression occurred in 5 patients during the follow-up period, and the median PFS was 19 months. CONCLUSION: Pineoblastoma is a rare central nervous system malignancy with a tendency for disseminated disease. Comprehensive therapies such as surgical resection, radiation and chemo therapy are effective therapies for PBs.