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目的:总结儿童多发性神经母细胞瘤(neuroblastoma,NB)的临床特征及生物学行为,以期对这类肿瘤的治疗及预后判断提供依据。方法:回顾性分析天津医科大学肿瘤医院儿童肿瘤科1995年1月至2015年12月收治的7例多发性NB患儿的临床资料并对其随访。结果:共收治360例NB患儿,其中多发NB仅7例(1.9%)。患儿平均年龄(19.4±11.9)个月;原发部位胸腹多发3例,双肾上腺多发4例;临床分期4S期4例(57.1%),3期2例(28.6%),4期1例(14.3%)。7例共15个原发灶:14个手术完整切除;组织学预后良好型(favorable histology,FH)12个(85.7%),组织学预后不良型(unfavorable histology,UH)2个(14.3%);13个(92.9%)MYCN基因无扩增。随访满3年的5例患儿,3年总生存率(overall survival rate,OS)为100%。结论:多发性NB极其罕见,具有初次诊断年龄较低、以4S期为主、手术易完整切除的临床特征,肿瘤细胞分化较成熟、FH居多、少见MYCN基因扩增等生物学行为。该病多数预后较好,应避免过度治疗。
Objective: To summarize the clinical features and biological behavior of children with multiple neuroblastoma (NB) in order to provide evidence for the treatment and prognosis of these tumors. Methods: The clinical data of 7 children with multiple NB admitted from January 1995 to December 2015 in Children’s Oncology Department of Tumor Hospital of Tianjin Medical University were retrospectively analyzed and followed up. Results: A total of 360 children with NB were treated, of whom 7 were multiple NB (1.9%). The average age of the children was (19.4 ± 11.9) months. There were 3 cases of primary pleural abdomen and 4 cases of double adrenal hyperplasia. The clinical stage was 4 (57.1%) in stage 4, 2 cases (28.6% Example (14.3%). There were 15 cases of primary tumor in 7 cases: complete resection in 14 cases; 12 histological favorable histology (FH) (85.7%), 2 unfavorable histology (UH) 2 (14.3% 13 (92.9%) MYCN genes were not amplified. Five children who were followed up for 3 years had a 3-year overall survival rate (OS) of 100%. Conclusions: Multiple nodules are extremely rare. They have the characteristics of younger primary diagnosis, 4S period and complete resection. The biological characteristics of the tumor are more mature, more FH and rare MYCN gene amplification. The prognosis of the disease is better, should avoid over-treatment.