Clinical diagnoses and treatments of neuroblastoma without an apparent primary lesion
Objective To explore the clinical features,diagnostic modalities and outcomes of neuroblastoma(NB)without an apparent primary tumor in children.Methods From June 2012 to February 2020,the relevant clinical data were retrospectively reviewed for 11 hospitalized NB children.There were 4 boys and 7 girls.NB was staged according to the International Neuroblastoma Staging System(INSS)and risk grouping was based upon the criteria of Children's Oncology Group(COG).After a definite diagnosis of NB,all children received stratified treatment according to the result of risk grouping.Follow-ups were conducted by outpatient examination and telephone interviews and the last follow-up was until April 21,2022.Result No primary tumor was detected in neck,mediastinum,posterior peritoneum,pelvic cavity or head.Median age of onset was 18.5(11.8,27.3)months.Initial symptoms included fever(n=4),abdominal distension(n=2),lower limb pain(n=2),fatigue &.poor appetite(n=1),jaundice(n=1),body surface mass(n=1)and orbital cyanosis(n=1).Metastases involved multiple bone marrow(n=7),liver(n=3)and back muscle(n=1).Neuron-specific enolase(NSE)was all positive(n=11),and 9 cases had an elevated level.Lactate dehydrogenase(LDH)was all positive(n=9),and 8 cases had an elevated level.And 24h urine vanillylmandelic acid(VMA)level was elevated in 5 cases.According to the INSS staging system,the stages were Ⅳ(n=9)and Ⅳs(n=2).Three cases receiving no tumor-related treatment and 1/8 treated cases died.The 5-year overall survival rate was 62.3％,the 5-year survival rate of children receiving tumor-related treatment 85.7％and the median survival 88 months.Conclusion An early diagnosis is difficult for NB without apparent primary tumor.Also its misdiagnosis and underdiagnosis are quite common.A definite diagnosis is dependent upon a pathological examination of metastatic lesions or bone marrow.After standardized treatment,a relatively decent therapeutic outcome may be obtained.
万方数据
维普
