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目的总结伴中枢神经系统及颅内侵犯的高危神经母细胞瘤(NB)患儿的临床特征,分析其预后影响因素。方法回顾性分析42例伴中枢神经系统及颅内侵犯的高危NB患儿的临床资料,并分析其预后影响因素。结果42例患儿临床表现主要有发热、肢体疼痛、突眼伴眶周淤青、下肢无力等。原发瘤为灶腹膜后来源33例,后纵隔来源8例。初诊36例,复发或进展8例。初诊36例患者中中枢神经系统转移3例,颅内转移33例,其中颅骨转移18例、硬脑膜受累21例、二者兼有16例。复发患儿以中枢神经系统复发多见。42例患儿5年无事件生存率为21.3%。颅骨受累是患儿预后不良的独立危险因素(P<0.01)。结论伴中枢神经系统及颅内侵犯的NB患儿预后较差,复发后再缓解率极低;颅骨受累是其预后不良的主要因素。
Objective To summarize the clinical features of high risk neuroblastoma (NB) patients with central nervous system and intracranial invasion and analyze the prognostic factors. Methods The clinical data of 42 high risk NB children with central nervous system and intracranial invasion were retrospectively analyzed. The prognostic factors were analyzed. Results The clinical manifestations of 42 cases were mainly fever, limb pain, exophthalmos with periorbital bruising, weakness in lower limbs and so on. The primary tumor was retroperitoneal source in 33 cases, followed by mediastinal source in 8 cases. The first visit in 36 cases, 8 cases of recurrence or progression. Among the 36 newly diagnosed patients, 3 had central nervous system metastasis, 33 had intracranial metastasis, 18 had skull metastasis, 21 had dura mater involvement, and both had 16 cases. Recurrent children with central nervous system recurrence more common. The 5-year event-free survival rate was 21.3% in 42 children. Skull involvement is an independent risk factor for poor prognosis in children (P <0.01). Conclusion The prognosis of NB patients with central nervous system and intracranial invasion is poor, and the remission rate is very low. The skull involvement is the main factor of poor prognosis.