小儿神经节细胞瘤的临床分析

Clinical analysis of gangliocytoma in children

目的探讨小儿神经节细胞瘤的诊断及治疗。方法结合相关文献对我科18例小儿神经节细胞瘤患儿的临床资料进行回顾性分析。男7例，女11例，年龄2～9岁，平均4岁。腹膜后8例，后纵膈6例，肾上腺4例。结合患儿临床表现及彩超、CT等影像学检查，14例术前考虑为神经节细胞瘤，2例误诊为神经母细胞瘤，1例误诊为嗜铬细胞瘤，1例误诊为肾母细胞瘤。结果18例患儿均行手术治疗，14例完整切除瘤体，2例椎管内残存有瘤体组织，2例仅行活检。随访17例，随访时间7d～7年，15例存活，2例死亡，2例复发。结论小儿神经节细胞瘤为起源于交感神经节的良性肿瘤，较为少见，发病年龄较大，瘤体生长缓慢，全身症状较轻，实验检查常无异常，完整切除后很少复发，术中应避免损伤腔静脉、腹主动脉，不可盲目处理伸向瘤体内部血管，如瘤体延伸至椎间孔内，为避免损伤神经根、脊髓，术后常有部分残余。

Objective To study the clinical characteristics of gangliocytoma in children. Methods In this study, the clinical data of 18 children with gangliocytoma were analyzed. The relevant litera- tures were reviewed. Among these patients, 8 cases were diagnosed with retroperitoneal gangliocyto- ma,6 were posterior mediastinal gangliocytoma, and the other 4 were adrenal gangliocytoma. Based on the preoperative ultrasonography and CT scanning, 14 cases were diagnosed as gangliocytoma, 2 were misdiagnosed as n＇euroblastoma, 1 was misdiagnosed as pheochromocytoma, and 1 was misdiagnosed as nephroblastoma. Results All patients underwent surgery. Among them, gangliocytoma was completely removed on 14 patients, and partly removed on 2. Two patients＂ tumors were failed to remove and biop- sy was performed to confirm the diagnosis. The patients were followed up for 7 day to 7 years. Fifteen patients survived,and 2 died. Gangliocytoma reoccurred on 2 patients. Conclusions Preoperative diag- nosis of gangliocytoma relies OR radiological study. Complete resection is the primary goal of surgery.