Clinical analysis of multi-disciplinary treatment for cervical neuroblastoma / 中华实用儿科临床杂志

ABSTRACT

Objective:To summarize the clinical features, treatment and outcomes of cervical neuroblastoma (NB) subjected to multi-disciplinary treatment.Methods:The clinical features of cervical NB patients who were admitted to Beijing Children′s Hospital, Capital Medical University from February 2015 to October 2018, were retrospectively analyzed.The tumor makers [lactate dehydrogenase(LDH), neuron-specific enolase(NSE), urine homovanillic acid/creatinine(HVA/Crn), and urine vanillyl-mandelic acid/creatinine(VMA/Crn)], index of tumor burden(KTB), bone marrow examination, histopathologic types, N- MYC, gene amplification and 11q23 depletion type, staging and grouping, treatment and outcomes were analyzed.Follow-up was ended on March 31 st, 2019. Results:The 13 cervical NB patients aged from 1 month to 47 months (median age 10 months), and 8 patients (61.5%) were younger than 18 months old.The course of disease ranged from 0.5 to 24.0 months (median course more than 1 month). Seven patients (53.8%) presented with cervical masses.According to International Neuroblastoma Staging System (INSS), 8 patients (61.5%) were identified as stage Ⅱ, 3 patients (23.1%) as stage Ⅲ, and 2 patients (15.4%) as stage Ⅳ.There were 8 patients (61.1%) at low risk, 4 patients (30.8%) at intermediate risk, 1 patient (7.7%) at high risk.As for the laboratory examinations, LDH was increased in 7 patients (53.8%), and normal in 5 patients (38.5%). NSE was increased in 9 patients (69.2%), and normal in 4 patients (30.8%). Urine VMA/Crn was increased in 5 patients (38.5%), and normal in 8 patients (61.5%). HVA/Crn was increased in 8 patients (61.5%), and normal in 5 patients (38.5%). KTB was increased in 5 patients (38.5%), and normal in 5 patients (38.5%). No NB cell was detected in bone marrow of 13 patients.The pathologic type was NB in 9 patients (69.2%), and ganglioneuroma in 4 patients (30.8%). N-MYC gene amplification and 11q depletion were not detected.All the 13 patients accepted regular chemotherapy, radiotherapy and primary tumor resection in accordance with the staging and clinical risk grouping.The range of follow-up time was 5 to 48 months (median 24 months). All of the patients fi-nished their treatment and were followed up regularly.Nine patients (69.2%) achieved complete remission, 4 patients (30.8%) achieved partial remission, and none of the patients had progression of disease.Conclusions:Cervical NB subjected to multi-disciplinary treatment has a smaller age at admission, mostly presented with cervical masses and rarely accompanied with distant metastasis.They are mostly at low risk or intermediate risk, and can achieve good outcomes after regular treatment.