Clinical and prognostic characteristics of neuroblastoma in children with abdominal and thoracic primary tumor sites

Abstract

Objective To explore the clinical, biological, relapsing and progression characteristics of neuroblastoma (NB) at abdominal and thoracic primary tumor sites. Methods A retrospective analysis of clinical characteristics was performed in 223 NB patients from January 2013 to January 2017. They were divided into abdominal (n=147, including adrenal and retroperitoneum) or thoracic (n=76) groups according to primary tumor sites. Comparative analyses were performed for clinical, biological, relapsing and progression features and survival curve was analyzed using SAS 9.4. Logistic regression analyses were performed for age, INSS (International Neuroblastoma Staging System) stage, pathological type, MYCN gene and primary sites of relapse and progression. Results The median age of abdominal and thoracic groups were 37 (24, 54) and 26 (14, 46) months respectively. AS compared with thoracic NB, abdominal NB had elevated levels of serum NSE (neuron specific enolase), ferritin, LDH (lactate dehydrogenase) and urinary HVA (homovanillic acid) (all P<0.05). Imaging findings revealed that maximal diameter of primary tumor in abdominal group was larger than that in thoracic group (median values of 11.30 vs 6.20 cm) and tumors in abdominal NB were more prone to encase essential blood vessels. According to the standards of INPC (International Neuroblastoma Pathology Classification System), unfavorable histopathology (UH) was found in 44.22% of abdominal group and favorable histopathology (FH) in 65.79% of thoracic group. MYCN amplification was detected in 23.13% of abdominal group and 1.32% of thoracic group. Patients with abdominal NB always had high risk disease with distant metastasis, meanwhile, thoracic NB patients tended to have a low/intermediate-risk disease and localized tumors. After a median follow-up of 29 months, 70 cases had tumor relapse or progression, including abdominal group (n=60) and thoracic group (n=10). Multivariate analysis revealed that MYCN gene amplification and primary abdominal site were significant independent predictors of relapse and progression. Conclusions NB has different clinical, biological, relapse and progression characteristics among different primary tumor sites. And abdominal NB is more frequently associated with poor prognosis. However, the prognosis of thoracic NB is fair. Key words: Neuroblastoma; Neoplasms by Site; Recurrence