儿童肾外肾母细胞瘤的诊断及治疗

Diagnosis and treatment of extrarenal Wilms'tumor in children

目的总结儿童肾外肾母细胞瘤有效的诊断方法及治疗方法。方法收集2020年1月至2021年10月就诊于山东第一医科大学附属省立医院的2例肾外肾母细胞瘤患儿的临床表现、实验室检查、影像学检查结果、治疗过程;检索PubMed、CHKD、CBM、万方和维普数据库中关于儿童肾外肾母细胞瘤的相关文献报道并进行回顾分析。结果患儿男1例,2岁2个月,因腹痛就诊,收住院后行超声及CT检查显示左侧腹膜后占位,考虑畸胎瘤,行腹腔镜左侧腹膜后肿瘤切除术,术后石蜡病理切片诊断为肾外肾母细胞瘤。患儿女1例,2岁11个月,因治疗发育迟缓,查体发现左侧腹股沟区包块待查来诊,术前行影像学检查显示左侧腹股沟区皮下肿物,考虑神经源性肿瘤,行左侧腹股沟区肿瘤切除术,术后石蜡病理切片诊断为肾外肾母细胞瘤。2例患儿均顺利出院,短期内随访,患儿恢复良好。检索发现26篇中英文文献,对其报道的44例患儿诊疗情况进行统计学分析,发现该病肿瘤所处位置有性别倾向(P=0.045),男性患儿好发于腹股沟区,女性好发于腹膜后;肿瘤所处的位置不同,所引起的临床症状也有所不同(P=0.017);患儿一年生存率为93.1%(27/29);通过生存分析(Breslow检验)患者生存时间的影响因素,肿瘤病理分期及病理组织学分型均是影响生存时间的独立因素(P<0.001),比较单纯手术治疗组与手术结合化疗组的生存时间,手术结合化疗患儿生存时间优于单纯手术(P=0.042)。结论肾外肾母细胞瘤临床罕见,目前主要依靠其临床表现及影像学检查辅助术前诊断,最终诊断依赖于组织病理及免疫组织化学,外科手术切除肿瘤是有效地治疗方案。

Objective To explore the effective diagnosis and treatment of extrarenal Wilms'tumor(WT)in children.Methods Clinical manifestations,laboratory examinations,imaging results and treatments were retrospectively reviewed for 2 extrarenal WT children.The databases of PubMed,China Hospital Knowledge Database(CHKD),China Biology Medicine(CBM),WanFang and VIP were searched for the related literature reports on pediatric extrarenal WT.Results A 26-month-old boy was hospitalized for abdominal pain.Ultrasound and computed tomography(CT)hinted at left retroperitoneal mass with a suspicion of teratoma.After laparoscopic tumor resection,extrarenal WT was confirmed by postoperative paraffin pathological examination.A 35-month-old girl was diagnosed as extrarenal WT by paraffin pathology.Due to growth retardation,a mass in left inguinal region was detected by physical examination.Preoperative imaging examination revealed subcutaneous mass in left inguinal region.Both cases recovered well after a smooth discharge.A total of 26 Chinese and English literatures were retrieved.The diagnoses and treatments of 44 children were analyzed.Tumor location had a gender tendency(P=0.045).Boys were inclined to occur in groin region while girls tended to occur in retroperitoneum.Clinical symptoms caused by different tumor locations varied greatly(P=0.017).The 1/2-year survival rate was 93.1%(27/29)and 77.8%(14/18).For those with local recurrence or distant metastasis,2-year survival rate was 50%(4/8)after treatment.Through survival analysis(Breslow test),the independent influencing factors of survival time were tumor pathological stage(P<0.001)and pathological classification(P<0.001).Survival time was better in operation and chemotherapy group than that in operation alone group(P=0.042).Conclusions Extrarenal WT is rare in clinical practices and its preoperative diagnosis is mainly aided by clinical manifestations and imaging examinations.Surgical tumor resection is the most effective treatment and a definite diagnosis depends on histopathology and immunohistochemistry.