关键词: 神经母细胞瘤, 临床特点, 预后, 婴儿

Abstract:

Objective The clinical characteristics, diagnosis and treatment characteristics and long-term curative effect of the children with neuroblastoma (NB) at the age of less than 12 months were analyzed to provide evidence-based basis for further optimizing the treatment of infantile NB patients. Methods The clinical data of infantile NB patients admitted from January 2008 to December 2018 were retrospectively analyzed, and the clinical characteristics and prognostic factors of the children were summarized. Results The median age of diagnosis in 51 NB infants (34 boys and 17 girls) was 7.5 (3.8-10.1) months, and abdominal mass (27 cases, 52.9%) was the commonest reason for medical treatment. The most frequently-occurring sites of tumors were adrenal gland (21 cases, 41.4%) and retroperitoneum (19 cases, 37.2%). Bone marrow, liver and bone were the commonest sites of metastasis. The amplification frequency of MYCN gene was 11.3% (5/44). Forty-nine NB patients underwent surgery, of whom 8 were treated with surgical resection alone, 13 were treated with surgery after chemotherapy, and 28 were treated with surgery before chemotherapy. Of the remaining patients, 1 received chemotherapy alone and 1 did not receive any treatment except follow-up. The median follow-up time was 78.5 (72.1-124.0) months. A total of 49 patients survived without an event. Two patients died. The 6-year overall survival rate and the 6-year event-free survival rate were both (96.1±2.7)%. Univariate analysis revealed that tumor staging, distant metastasis, bone marrow metastasis, bone involvement, risk grouping, MYCN amplification, serum neuron-specific enolase and lactate dehydrogenase levels at initial diagnosis were factors affecting prognosis (P<0.05). Conclusions Infantile NB patients have a good long-term prognosis. It should be expected to further reduce the intensity of chemotherapy for patients without MYCN amplification.

Key words: neuroblastoma, clinical characteristic, prognosis, infant