摘要目的 探讨小儿恶性蝾螈瘤(malignant triton tumor,MTT)的临床表现、病理特征、诊疗方法及术后随访情况,并提供系统的诊疗方案,降低误诊率及漏诊率,延长患儿生存时间.方法 我院自2000年至2013年共收治小儿恶性蝾螈瘤3例,均经病理证实.女2例,男1例,年龄分别为4、6、12岁.3例均为首次就诊,2例肿物位于腹膜后,1例位于阴囊内.3例患儿均以发现无痛性肿物为代主诉入院,1例伴乏力、腹胀,其余2例均无明显临床症状.3例术前均行CT平扫及增强扫描.3例患儿手术过程均较顺利,肉眼所见肿瘤均完整切除,术中探查均未发现其他部位可疑病灶.结果 3例术后恢复良好.1例术后未进行辅助治疗,术后3年出现右下肢疼痛,右下肢皮肤无汗,彩超及MRI均提示右下肢占位,考虑复发,患儿家属拒绝再次手术.1例术后采用VAC化疗方案,定期复查CT未发现明显异常,已无瘤生存2年.1例术后1个月病理确诊为恶性,加行腹股沟及盆腔淋巴结清扫术,术后采用VAC和OPEC化疗方案交替进行,共化疗15次,现已无瘤生存4年.目前此2例情况良好,无复发征象.结论 恶性蝾螈瘤属高度恶性,术后易复发及转移,确诊仍需依靠组织病理学及免疫组化分析,手术为唯一首选治疗方法,术后辅助化疗可在一定程度上改善患儿预后.

abstractsObjective To explore the clinical characteristics,histopathological results,diagnosis and treatment of childhood malignant triton tumor (MTT) and formulate a systematic diagnostic and therapeutic scheme for decreasing the rate of misdiagnosis and prolonging the survival time.Methods The clinical data of 3 children with a definite diagnosis of MTT between 2000 and 2013 were analyzed.There were 2 girls and 1 boy aged 4,6 and 12 years respectively.All had initial presentations.The tumor sites were retroperitoneal (n =2) and scrotum (n=1).Painless lump was a chief complaint.One case had malaise and abdominal distension while the remainder showed no apparent clinical symptoms.All operations went smoothly.Macroscopic tumors visible were removed completely without any finding of suspicious lesions.Results Postoperative recoveries were all excellent.One case refused postoperative chemotherapy and right low extremity became painful and anhidrotic at 3 years post-operation.Color duplex ultrasonography and magnetic resonance imaging (MRI) showed an occupancy lesion in right lower extremity.The relapsing child's family refused reoperation.One case received chemotherapy regimen of VAC.And there was no obvious abnormality on abdominal computed tomography (CT) and survived tumor-free for 2 years.Another case was confirmed by pathology.At 1 month post-operation,inguinal and pelvic lymph node dissections were performed.Alternating chemotherapy regimens of VAC and OPEC were offered for 15 cycles.The tumor-free survival was 4 years.Up to now,two cases showed no sign of relapse.Conclusions Malignant triton tumor is highly aggressive and susceptible to recur and metastasize.And a definite diagnosis is dependent upon histopathology and immunohistochemistry.Surgery is a first-choice option.And adjuvant chemotherapy and radiotherapy after operation may improve patient prognosis.