LINC-14. A single center retrospective analysis of pediatric pineoblastoma in Beijing

OBJECTIVE: To explore the clinical characteristics and outcome in children with pineoblastoma in Beijing. METHODS: Clinical data of 18 pediatric patients with newly diagnosed pineoblastoma admitted to Beijing Shijitan Hospital between January 2014 and November 2021 were retrospectively analyzed. The diagnoses were confirmed by pathology. RESULTS: Male/female ratio=8:1. The median age at diagnosis was 4.7 (range, 0.2-12.6) years, with 2 cases in infancy, and 13 cases ≥ 3 years. The symptoms at diagnosis included headache (31%), vomiting (29%), convulsions (9%), strabismus (9%), nausea (6%), etc. Four patients experienced metastasis at diagnosis. Ki-67 index was under 30% in 5 cases, 30-80% and ≥80% in 10 and 2 cases, respectively. All were treated with surgery, and 12 children underwent gross total resection (GTR). Seventeen cases were administered both radiotherapy and chemotherapy, with one case only radiotherapy followed by surgery. Median follow-up time was 54 months. Nine patients developed a recurrence and 2 patients died at last follow-up. The 1-year/3-year progression-free survival (PFS) and overall survival (OS) were (77.8±10.5/11.1±10.5)%, and (100/90.9±8.7) %, respectively. The 3-year OS of boys (93.8%) was higher than that of girls (50.0%); and also higher in cases with GTR (91.7%) than STR (83.3%). However, the differences were not significant in the above two groups. The children with Ki-67 index ≥80% had worse 3-year OS than those<80% (χ2=8.000, P=0.005). The median PFS of children treated under the order of craniospinal irradiation followed by chemotherapy was better than that of the inverse order (29m vs 13m, χ2=6.528, P=0.011).CONCLUSION: Pineoblastoma is rare and often fatal, but with better OS in our center, although the PFS is dismal. Boys, GTR resections, and Ki-67 index <80% tends to have better OS, and the order of irradiation followed by chemotherapy tends to have better PFS. Keywords: pineoblastoma; therapy; survival