肾上腺外嗜铬细胞瘤的诊断与治疗(附18例报告)

目的 探讨肾上腺外嗜铬细胞瘤的诊断和治疗方法。方法 回顾性分析18例肾上腺外嗜铬细胞瘤的临床资料。男13例，女5例。平均发病年龄30岁。18例均有不同程度的血压升高，头痛头晕(67％)、心悸多汗(44％)、视物不清(33％)及肉眼血尿(28％)为主要临床症状。尿儿茶酚胺(CA)和(或)3-甲氧4—羟苦杏仁酸(VMA)阳性者16例，阳性率89％。经B超、CT、MRI及膀胱镜等检查，肿瘤位于膀胱7例、腹主动脉旁5例、肾门4例、脐尿管1例、盆腔1例。结果 18例中行肿瘤切除术15例，探查术3例。病理诊断良性16例，恶性2例。5例术后3个月～10年复发。结论 尿CA和VMA测定是定性诊断和监测复发的有效方法。围手术期控制血压和扩容是保障手术安全的关键。术后应长期严密随访。

Diagnosis and treatment of extra-adrenal pheochromocytoma (report of 18 cases)

Objective To study the diagnosis and management of extra-adrenal pheochromocytoma. Methods Eighteen patients with extra-adrenal pheochromocytoma were analyzed retrospectively.Among the 18 patients 13(72%) were men.The mean age at diagnosis was 30 years,with a range from 15 to 65 years.All the patients presented with hypertension.Other typical symptoms included headache and dizziness(67%),palpitation and sweating(44%),blurred vision (33%) and hematuria (28%).Of all the patients 89% were diagnosed with urine CA and VMA.Most of the tumors were located at the bladder (39%),para-aorta (28%) and renal hilus(22%). Results All the patients were treated with surgical operation including 14 total resection of tumor,1 simple resection of tumor and 3 biopsy.Two of the 18 cases were malignant according to the post-operative pathologic diagnosis.Five patients recurred 3 months to 10 years after operation. Conclusions The determination of CA and VMA in urine is valuable for the diagnosis and monitoring of postoperative recurrence.Ultrasonography and CT scanning are effective means for tumor localization.The perioperative management of controlling blood pressure and expansion of the blood volume are very important.Surgical extirpation is a good method for the effective treatment.Postoperative long-term follow up is necessary.