儿童肾恶性横纹肌样瘤并发放射性肝损伤1例并文献复习  

A case report of a child with a malignant rhabdoid kidney tumor complicated with radiation-induced liver injury and literature review

作　　者：廖雪莲 蒋莎义 刘江斌 吴滢[3] Liao Xuelian;Jiang Shayi;Liu Jiangbin;Wu Ying(Department of Hematology and Oncology,Shanghai Children's Hospital,School of Medicine,Shanghai Jiao Tong University,Shanghai 200062,China;Department of General Surgery,Shanghai Children's Hospital,School of Medicine,Shanghai Jiao Tong University,Shanghai 200062,China;Department of Pathology,Shanghai Children's Hospital,School of Medicine,Shanghai Jiao Tong University,Shanghai 200062,China)

机构地区：[1]上海市儿童医院,上海交通大学医学院附属儿童医院血液肿瘤科,上海20006 [2]上海市儿童医院,上海交通大学医学院附属儿童医院普外科,上海200062 [3]上海市儿童医院,上海交通大学医学院附属儿童医院病理科,上海200062

出　　处：《国际放射医学核医学杂志》2024年第8期531-534,F0003,共5页International Journal of Radiation Medicine and Nuclear Medicine

基　　金：上海市儿童医院教学课题(2020KC05)。

摘　　要：笔者报道了1例12月龄女性右肾恶性横纹肌样瘤Ⅱ期患儿,右肾肿瘤根治术后瘤床接受放疗,照射累积剂量19.5 Gy/13 Tx,治疗后2个月出现厌食、腹泻和肝功能损伤,Child-Pugh评分为5分A级,腹部CT显示肝左叶密度异常,不均匀,边界清晰。手术切除肝左叶病变,组织病理学检查显示以肝静脉窦受损和肝细胞气球样变性为主,未见肿瘤细胞,诊断为典型的放射性肝损伤(RILD)。经护肝治疗并继续完成化疗,2个月后肝功能和腹部CT检查结果均见好转。随访36个月无病生存。通过该案例分析和文献复习,分享儿童典型的RILD临床特征,影像学上肝脏病变范围与受照范围一致,呈刀切样,组织病理学检查结果以肝静脉闭塞性改变为主。儿童RILD经护肝治疗,临床可恢复。The author reported a 12-month-old female child with stageⅡmalignant rhabdoid tumor of the right kidney.The tumor bed received radiotherapy after radical resection of the right kidney tumor.The cumulative dose of irradiation was 19.5 Gy/13 Tx.Anorexia,diarrhea and liver function injury occurred 2 months after treatment.The child's liver function was impaired with A Child-Pugh score of 5,grade A,and abdominal CT showed abnormal density,uneven,and clear boundaries in the left lobe of the liver.After surgical resection of the left lobe of the liver,histopathological examination revealed hepatic venous sinus damage and balloon degeneration of hepatocytes,and no tumor cells were found,which was diagnosed as classic radiation-induced liver disease(RILD).The trearment team used drugs to protect RILD and continued to complete chemotherapy,and both liver function and abdominal CT improved after 2 months.Up to 36 months of follow-up,there was no disease survival.Through the case analysis and literature review,the classic clinical features of RILD in children were shared.The scope of liver lesions on imaging was consistent with irradiation range,showing changes like a knife cut,and the histopathological findings were mainly hepatic venous occlusion.RILD in children can be recovered clinically after liver protection treatment.

关 键 词：放射性肝损伤 护肝治疗 肝左叶 肝功能损伤 组织病理学检查 无病生存 腹部CT 累积剂量 

分 类 号：R737.11[医药卫生—肿瘤] R818[医药卫生—临床医学]