家族性嗜铬细胞瘤一家系诊疗及文献复习  

Diagnosis and treatment of familial pheochromocytoma in a kindred and literature review

作　　者：陈红兵 程冬 周正兴 朱敬波 李清华 梁朝朝[2] 

机构地区：[1]安徽医科大学第三附属医院泌尿外科,合肥230061 [2]安徽医科大学第一附属医院泌尿外科

出　　处：《现代泌尿生殖肿瘤杂志》2015年第1期31-34,共4页Journal of Contemporary Urologic and Reproductive Oncology

摘　　要：目的探讨家族性嗜铬细胞瘤的遗传特征、临床表现及治疗方法。方法结合文献回顾性分析一家系家族性嗜铬细胞瘤患者的临床资料。结果该家系两代人中有4例肾上腺嗜铬细胞瘤患者,均在青少年期发病,其中1例为双侧发病。采用肾上腺肿瘤切除术治疗。病理诊断为良性嗜铬细胞瘤,随访6个月至28年无复发。结论家族性嗜铬细胞瘤符合常染色体显性遗传特点,多在青少年期发病。目前尚无预防手段,早期诊断和长期随访是提高治愈率的唯一方法。Objective To describe the clinical presentation,outcome of surgery and genetic characteristics of familial pheochromocytoma. Methods We retrospectively analyzed the clinical data of a pheochromocytoma family combined with relevant literature review. Results There were four members with pheochromocytoma in the family of two generations.All tumors occurred in adolescence which were planted on the adrenal body,only 1patient with bilateral tumors.All four patients received operation of adrenalectomy.The pathologic results showed benign adrenal pheochromocytoma.The follow-up lasted for 6months to 28 years without recurrence. Conclusions Familial pheochromocytoma belongs to autosomal dominant inheritance disease.The tumor occurs mostly in adolescence.No specific treatment has been proposed for prevention of this disease.Thus,early diagnosis and regular follow-up are the only means for a better outcome.

关 键 词：家族性嗜铬细胞瘤 诊断 治疗 

分 类 号：R736.6[医药卫生—肿瘤]