Characterizing Prognostic Factors in Adult-Onset Neuroblastoma: A Population-Based Study [post]

Yao Zhang, Chenxuan Yang
2021 unpublished
Background: Neuroblastoma (NB) is the most commonly diagnosed malignancy among pediatric patients with a high level of heterogeneity. NB is rarely seen in adult patients, yet adult-onset NB is associated with poor outcome and distinct clinical features from pediatric NB. Guidelines for the management of adult-onset NB are not yet defined and the treatment is largely dependent on the experience of corresponding oncologists. Previous reports on adult-onset NB have mostly been case reports or case
more » ... series, without high-quality, population-based cohort study. Our study aims to provide new insights and promotes understanding of adult-onset NB using the data from Surveillance, Epidemiology and End Results (SEER) program.Methods: We extracted data of primary NB patients diagnosed by positive histology during 1975-2016 from the SEER database, and grouped the patients according to the age of diagnosis. Competing-risk model and Kaplan-Meier plot were used to compare survival rates of adult-onset neuroblastoma with pediatric neuroblastoma. Cox proportional hazard regression model was used in univariate and multivariate analysis to identify prognostic factors affecting overall and cancer-specific survival rates in adult patients. A nomogram was developed according to the multivariate model and internally validated using receiver operator curve (ROC) and decision curve analysis (DCA).Results: We found a worsened outcome (Median survival time 31 vs 70 months) and distinct clinical characteristics in adults compared with adolescents. Univariate analysis revealed that age (HR=1.7627, 95%CI: 1.1937-2.6027, p = 0.004), surgery (HR=0.3968, 95%CI: 0.2691-0.5852, p <0.0001), primary tumor site, histology type (HR=1.9792, 95%CI: 1.1159-3.5103, p = 0.0195), chemotherapy (HR=1.5723, 95%CI: 1.0284-2.4037, p = 0.0367) was significantly associated with prognosis. Upon multivariate Cox proportional hazard regression model, age, surgery, primary tumor site remained significant for cancer-specific survival. Using the multivariate regression model, we proposed a new nomogram for the prediction of 3-year and 5-year survival rate. The nomogram showed good performance on survival prediction and risk-group stratification in an internal validation cohort. Conclusion: In conclusion, our study provided novel evidence on prognosis of adult-onset NB using large-scale populational database and identified surgical intervention as the most effective treatment toward adult-onset NB. Our nomogram can serve as potential prediction and risk-stratification tool in clinical practice.
doi:10.21203/rs.3.rs-198536/v1 fatcat:iariy6sj2jewnnabmmec54s2k4