110例儿童神经母细胞瘤临床特征及预后分析

Analysis of Clinical Characteristics and Prognosis of 110 Pediatric Patients with Neuroblastoma

【目的】提高对儿童神经母细胞瘤临床特征及预后的认识。【方法】回顾性分析本院2008年3月至2012年9月资料完整的1】O例儿童神经母细胞瘤患儿的临床表现、肿瘤标志物、病理类型、治疗、并发症及转归。【结果】男60例，女50例，中位年龄32个月，原发肿瘤位于肾上腺42例，腹膜后37例，纵隔26例，盆腔3例，颈部2例。初诊时腹部神经母细胞瘤以发热和（或）腿痛，腹痛、腹胀，包块多见，纵隔、颈部神经母细胞瘤以咳嗽及神经系统表现常见。3年总生存率为66％。初诊年龄〉18个月（P〈0．01）、Ⅲ和Ⅳ期（P=0．008）、纵隔以外原发肿瘤（P=0．027）、血清神经元特异性烯醇化酶（NSE）〉100ng／L（P=0．01）及手术切除程度（P〈O．01）是预后不良的指标，而血清NSE及肿瘤未能完整切除是独立的预后影响因素。【结论】多数神经母细胞瘤初诊时有临床表现，但不特异，掌握其临床特征早期确诊、完整切除原发肿瘤有利于改善预后；年龄〈18个月者降低化疗强度值得推荐。

[Objective] To improve the understanding of clinical features and prognosis of pediatric neuro- blastoma. [Methods] Clinical manifestations, tumor markers, pathological types, treatment, complications and outcomes of 110 pediatric patients with neuroblastoma with complete data from March 2008 to Sept. 2012 were analyzed retrospectively. [Results] Of 110 patients, 60 patients were male and 50 patients were female. The median age was 32 months. Primary tumors of 42, 37, 26, 3 and 2 patients located in adrenal gland, ret- roperitoneum, mediastinum, pelvic cavity and neck, respectively. Most initial manifestations of abdominal neuroblastoma were fever and/or leg pain, abdominal pain, abdominal distention and masses. Cough and neu- rological syndromes were commonly seen in mediastinal and cervical neuroblastoma. The 3-year overall survival rate was 66 ~. Poor prognostic factors included the onset age^18 months（ P d0. 001）, stage 11I and IV （ P = 0. 008 ）, primary tumors except mediastinal tumor（ P =0. 027 ）, serum NSE^100 ng/L（ P d0. 001） and operation resection extent（ P d0. 001）. And serum NSE and incomplete resection of tumor were independent factors affecting the prognosis. [Conclusion] Most of neuroblastoma at the first diagnosis have clinical manifes- tations, but they are not specific. The mastering of clinical features, early diagnosis and complete resection of primary tumor are helpful for improving the prognosis. The intensity of chemotherapy can be reduced for pa- tients with the age lower than 18 months. It is worth to be recommended.