八例小儿先天性中胚层肾瘤的临床分析

Eight cases of congenital mesoblastic nephroma： clinical analysis and literature review

目的提高对小儿先天性中胚层肾瘤的认识。方法回顾性分析我院2004年4月至2012年8月收治的8例先天性巾胚层肾瘤患儿的年龄、性别、发病部位、临床症状、病理特征、影像学特征和治疗方案及预后。结果8例患儿均接受手术治疗，4例全肾切除，1例半肾切除，3例肿瘤核除。病理分类：经典型2例，细胞型6例。术前化疗2例，术后化疗3例。1例肿瘤核除术后8个月复发接受二次手术。B超和CT提示实质性占位3例，经典型2例；含囊性／钙化／坏死5例，皆为细胞型。8例患儿2例失访，6例无瘤存活至今，平均随访时间14．6个月，最长36个月。结论先天性中胚层肾瘤是一种来源于中胚叶组织的良性肿瘤，诊断困难，影像表现与病理分型相关，治疗以手术为主，术前化疗不敏感，术后化疗作用有待进一步研究，预后良好。

Objective To heighten the realization of congenital mesoblastic nephroma （CMN） in children. Methods Eight cases of CMN treated in our hospital from Apr. 2004 to Aug. 2012 were included in this study. The age, gender, the location of pathologic change, clinical symptoms, pathological characteristics, imaging characteristics, therapeutic regimen and prognosis were analyzed retrospectively. Results Surgical treatment were performed in all eight patients, which including whole renal resection for 4 cases, tumor resection for 3 cases and half nephrectomy for l case. Pathological classification revealed classic CMN in 2 cases and cellular CMN in 6 cases. Two cases received preoperative chemotherapy but no response while 3 cases received postoperative chemotherapy. Three cades of classic CMN were demonstrated as solid mass while 5 cases of cellular CMN were demonstrated as focal areas of cystic/necrosis/calcification by ultrasound and CT scan. The mean follow up time was 14. 6 months, the longest was 36 months. Two cases lost to follow up, tumor recurrence occurred in 1 case at the eighth month after operation and surgical resection performed again. Six patients survived uneventfully. Conclusions CMN is a kind of rare benign tumor which comes from mesoderm, differential diagnosis should be made with Wilms＇ tumor; imaging characteristics have part correlation with pathologic characteristics; surgery is the main treatment, preoperative chemotherapy is not sensitive, the effect of postoperative chemotherapy need further study, the prognosis is fairly good.