摘要
目的探讨肾移植术后延胡索酸水合酶缺陷型肾细胞癌的临床特征,提高该疾病的临床诊治水平。方法本研究为回顾性研究,选取2022年3月陆军特色医学中心泌尿外科经治的1例肾移植术后延胡索酸水合酶缺陷型肾细胞癌患者作为研究对象,收集其临床资料,包括临床特点、治疗方案和预后,并复习国内外文献,针对该病例的特点进行分析和总结。结果患者女性,年龄52岁,20年前接受肾移植,诊断为右侧原肾肾癌伴远处转移,后接受减瘤性肾切除术,病理诊断为延胡索酸水合酶缺陷型肾细胞癌(根据2022版WHO肾脏肿瘤分类的修正诊断)。术后予以靶向药物治疗后因血肌酐升高停药,病情进展。结论延胡索酸水合酶缺陷型肾细胞癌进展快、预后差,诊断依赖免疫组化及基因检测。目前肾移植术后的肾癌缺乏有效的治疗手段,针对此类患者应选择个体化治疗方案。
Objective To investigate the clinical characteristics of fumarate hydratase-deficient renal cell carcinoma(FH-RCC)in kidney transplant patients so as to improve the diagnosis and treatment of the disease clinically.Methods We retrospectively analyzed and summarized the clinical data of one kidney transplanted patient with FH-RCC who was treated in the urological department of our medical center in March 2022,including clinical characteristics,treatment and prognosis.A literature review was also performed to analyze and summarize its characteristics.Results A 52-year-old female patient who received a kidney transplant 20 years ago was diagnosed with right kidney cancer with multiple metastases.Cytoreductive nephrectomy was performed and pathological type was FH-RCC based on Revised Version of WHO 2022 Classification of Renal Tumors.She then received targeted therapy until discontinuation due to elevated serum creatinine and the disease progressed rapidly.Conclusion FH-RCC is characterized by rapid progress and poor prognosis.Its diagnosis relies on immunohistochemistry and genetic test.There are no currently standard-of-care treatment options for kidney transplant patients with renal cell carcinoma.Individualized treatment regimens should be suggested for these patients.
作者
汪月明
马强
刘高磊
钟晓
张尧
兰卫华
江军
舒泽华
WANG Yueming;MA Qiang;LIU Gaolei;ZHONG Xiao;ZHANG Yao;LAN Weihua;JIANG Jun;SHU Zehua(Department of Urology,Army Medical Center of PLA,Chongqing,400042,China;Department of Pathology,Army Medical Center of PLA,Chongqing,400042,China)
出处
《陆军军医大学学报》
CAS
CSCD
北大核心
2023年第13期1436-1440,共5页
Journal of Army Medical University
关键词
延胡索酸水合酶缺陷型肾细胞癌
肾移植
诊断
治疗
fumarate hydratase-deficient renal cell carcinoma
kidney transplant
diagnosis
treatment
