异位嗜铬细胞瘤患者23例临床分析

目的 总结第二军医大学附属长海医院收治的23例异位嗜铬细胞瘤患者，提高临床医生对该病诊断、治疗的认识。 方法 回顾性分析第二军医大学附属长海医院收治的23例经病理证实的异位嗜铬细胞瘤患者，总结其临床特点、治疗方法及预后。 结果 获取了1991年5月—2016年3月于第二军医大学附属长海医院诊治的23例异位嗜铬细胞瘤患者资料，其中女性10例(43.5%)，男性13例(56.5%)；平均发病年龄50.4岁。异位嗜铬细胞瘤的典型表现为儿茶酚胺样症状，最常见的症状分别为头痛7例(30.43%)，心悸5例(21.7%)，腹部隐痛及腹部不适5例(21.7%)，肉眼血尿2例(8.7%)，大汗1例(4.3%)，另有10例(43.7%)患者为隐匿症状。本组异位嗜铬细胞瘤均位于腹膜后，其中14例(60.9%)位于腹主动脉及下腔静脉旁，4例(17.3%)位于肾门附近，3例(13.0%)位于胰头，2例(8.7%)位于膀胱。CgA免疫组化染色是主要的病理确诊方法。23例患者全部施行手术治疗，行开放式手术的20例(87.0%)，腹腔镜辅助的1例(4.3%)；2例膀胱异位嗜铬细胞瘤患者均施行经尿道膀胱肿瘤电切术。大部分异位嗜铬细胞瘤的预后较好，5年生存率95.6%。2例恶性者术后复发，其中1例于术后25个月发生腹腔转移，并于术后40个月死亡。 结论 异位嗜铬细胞瘤是一种发病率极低的肿瘤，多位于腹主动脉、下腔静脉旁，可伴高血压以及头痛、心悸等临床表现，亦有部分隐匿症状者。主要的治疗方法为手术切除。该病预后普遍较好，部分恶性患者预后较差。 

Clinical analysis of 23 cases of ectopic pheochromocytoma

Objective To summarize the clinical data of 23 ectopic pheochromocytomas collected in Changhai hospital,and to improve the diagnosis and management of this disease. Methods The clinical data of 23 cases of pathologically proven ectopic pheochromocytomas in our hospital were retrospectively reviewed and systematic followed up. Results Total 23 cases of pathologically proven ectopic pheochromocytomas in our hospital between May,1991 and March,2016,10 female patients and 13 male patients with a median age of 50.4 years,were enrolled into this study.The manifestations of ectopic pheochromocytomas were usually typical.Headache was shown in 7 cases(30.43%),palpitations was shown in 5 cases(21.7%),abdominal pain was shown in 5 cases(21.7%),painless hematuria was shown in 2(8.7%),and sweating was shown in 1 cases(4.3%),and other 10 cases of patients without any symptoms(occult occurrence).All of tumors were located in retroperitoneum.The anatomic locations of the ectopic pheochromocytomas were adjacent to the abdominal aorta and the inferior vena cava in 14 cases(60.9%),adjacent to the renal hilum in 4 cases(17.3%),and adjacent to the head of pancreas in 3 cases(13.0%).Other 2 cases were located in urinary bladder.CgA immunohistochemisty was a significant criterion in pathological diagnosis in our cases.All of the 23 of patients conducted surgical excision.Twenty(87.0%) of them were open surgeries,one cases conducted laparoscopic surgeries,and 2 cases conducted transurethral resection of bladder tumor.Most of the benign patients remained free from recurrence with good prognosis,while 2 malignant cases presented with poor prognosis. Conclusion Ectopic pheochromocytoma is a kind of rare tumors,usually located in retroperitoneum,and adjacent to the abdominal aorta and the inferior vena cava.Most cases have good prognosis,and surgical excision is the mainly treatment.