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目的分析双侧肾上腺嗜铬细胞瘤(PHEO)的临床特点,提高临床医师对该病的认识。方法采用回顾性病例分析方法,总结北京协和医院1952-2004年收治的25例双侧肾上腺PHEO病例的临床诊治资料。结果双侧肾上腺PHEO患者诊断年龄较轻,(32±14)岁;76%(19例)的双侧肾上腺PHEO为家族性PHEO;52%为多发性内分泌腺瘤病,20%为vonHippel-Lindau病;88%的病例(22例)双侧病变同时被发现,双侧肾上腺中至少一侧为多个肿瘤者占56%,并且50%的PHEO术后复发。结论对双侧肾上腺PHEO病例需检查其他内分泌腺体是否受累,并对其家族成员进行筛查,手术时注意可能存在多发肿瘤,术后需长期随访。
Objective To analyze the clinical features of bilateral adrenal pheochromocytoma (PHEO) and to improve clinicians’ understanding of the disease. Methods A retrospective case analysis was conducted to summarize the clinical data of 25 patients with bilateral adrenal PHEO admitted from 1952 to 2004 in Peking Union Medical College Hospital. Results Patients with bilateral adrenal PHEO had a younger age of diagnosis (32 ± 14) years; 76% (19 cases) of bilateral adrenal PHEO were familial PHEO; 52% were multiple endocrine neoplasia; 20% were von Hippel-Lindau 88% of cases (n = 22) were also found with bilateral lesions, with 56% of multiple tumors in at least one side of the adrenal gland and 50% of PHEO recurrences. Conclusions The bilateral adrenal PHEO cases need to check whether other endocrine glands are involved and their family members are screened. At the time of surgery, there may be multiple tumors, and long-term follow-up is needed after the operation.