腹膜后恶性蝾螈瘤2例报告

目的：总结恶性蝾螈瘤（malignant triton tumor，MTT）的临床及病理特点、诊治方法及预后。方法：回顾性分析2例腹膜后MTT患者的临床资料，均为女性，分别为8个月及44个月大婴幼儿。均因体检发现腹部肿物入院。下腹部均可触及质韧肿物，表面光滑，活动度可，无压痛。B超、CT、MRI等影像学检杏见中下腹或腹盆腔区有大小约14cm×12cm×9cm和15cm×13cm×10cm肿块，增强CT示肿物呈不均匀强化。2例患者均行肿瘤根治性切除术。结果：镜下见肿瘤由梭形细胞及多角形细胞组成，可见病理性核分裂，伴有横纹肌分化。免疫组织化学染色示NSE＋，Vimentin＋，Desmin＋，S-100＋。诊断为腹膜后MTT。1例患者随访3个月，术后2个月复发转移，近期肿物有所增长。另1例随访5个月，目前情况良好。结论：腹膜后MTT是一种罕见、高度恶性的肿瘤，确诊需依赖病理检查和免疫组织化学检测。本病以手术根治治疗为主，术后易复发和转移，预后差。

Retroperitoneal Malignant Triton Tumor:2 Cases Report

Objective:To discuss the clinical and pathological features,diagnosis,management and prognosis of retroperitoneal malignant triton tumor.Method:2 cases of retroperitoneal malignant triton tumor were reported and the relevant literature was reviewed.The ages of 2 cases were 8 and 44 months.They were admitted with abdominal mass.The mass was palpable and without tenderness during physical examination,B ultrasound,CT and MRI revealed 14 cm×12 cm×9 cm and 15 cm×13 cm×10 cm mass exhibiting inhomogeneous enhancement located at inferior abdomen and abdominopelvic cavity,2 patients were all taken retroperitoneal tumor resection.Result: The diagnosis of retroperitoneal malignant triton tumor in the patient was confirmed by postoperative pathology. Under microscope,tumor was consisted by spindle and polygonal cells with abundance cytoplasm.Pathologic fission was visible accompanied by nonstriated muscle differentiation.Immunohistochemical staining showed that the tumor were positive for the markers NSE,Vimentin,Desmin,S-100,but Actin was negative.1 case was followed up for 3 months,and the patient was considered local recurrence and multi-metastasis at 2 moths after the surgery,unfortunately,the tumor was in growing condition.The other patient was followed up for 5 months and was in good condition.Conclusion:Retroperitoneal malignant triton tumor is extremely rare with a high grade of malignancy,and its prognosis is poor.The diagnosis depends on pathological features and immunohistochemical staining results.Radical resection is considered to be the most reliable treatment so far.