Incidence, Treatment and Survival in Primary Central Nervous System Neuroblastoma.

BACKGROUND: Primary central nervous system neuroblastoma (PCNSN) is a rare disease, and its incidence, treatment modalities, and survival remain poorly understood. METHODS: The SEER database was used to identify patients diagnosed with PCNSN from 1973 to 2013. The incidence and survival rates were examined. Clinical features, treatment modalities and prognosis were also assessed. RESULTS: 280 PCNSN patients were identified, with annual age-adjusted incidence being 0.37 per 1,000,000 persons in 1973 and decreasing to 0.12 in 2013. Neuroblastoma (NBL) (ganglioneuroblastoma [GBNL] vs NBL; OR, 25.01; P=0.008) and tumor with distant metastasis (odds ratio [OR], 0.17; P=0.002) were more likely to receive conservative treatment over surgery, while older age (OR, 1.02; P=0.011) and tumors located in brain (Other nervous system vs. brain: OR, 0.31; P=0.001) increased the likelihood of receiving combined surgery and radiotherapy over surgery alone. In addition, younger age, GNBL, and surgery treatment were significantly associated with improved outcomes (all P<0.05). Furthermore, a nomogram model was established to effectively estimate survival rates for PCNSN patients. CONCLUSIONS: We updated epidemiological information of PCNSN and demonstrated that age, histological type, tumor extension and surgery were independent prognostic factors. Moreover, treatment modalities of these tumors are influenced by patient and tumor characteristics.