肾上腺组合性嗜铬细胞瘤四例报告及文献复习

目的 探讨肾上腺组合性嗜铬细胞瘤(嗜铬细胞瘤-神经节瘤)的临床特点及治疗方法.方法病理确诊的肾上腺组合性嗜铬细胞瘤患者4例,均为男性,年龄37～62岁.临床表现:高血压3例,其中伴有阵发性心悸1例;血压正常1例.B超、CT检查提示肿瘤均为单发,直径分别2.5、3.8、6.3、15.0 cm.行131Ⅰ-间位碘代苄胍显像检查2例,均为阳性;99Tcm-奥曲肽显像检查2例,阳性1例.24 h尿儿茶酚胺检查,去甲肾上腺素(196.1±92.2)nmol/24 h,肾上腺素(26.6±8.9)nmol/24 h,多巴胺(1957.9±913.5)nmol/24 h,其中轻度升高2例,正常2例.术前α受体阻滞剂准备2～4周.结果 3例行后腹腔镜下肾上腺肿瘤切除手术,1例行开放手术切除.病理报告:肾上腺组合性嗜铬细胞瘤.4例随访15～38个月,平均28个月,肿瘤未见复发、转移;3例高血压患者中2例血压正常.结论 肾上腺组合性嗜铬细胞瘤临床罕见,临床表现与嗜铬细胞瘤相似,儿茶酚胺稍升高,无恶性倾向,手术效果良好.

Composite pheochromoeytoma of adrenal: report of 4 cases

Objective To discuss the clinical characteristics and management principles of com-posite pheochromoeytoma of adrenal gland (pheochromocytoma-ganglioneuroma). Methods Four cases of composite tumor of adrenal gland diagnosed pathologically were reviewed. All the cases were male, aged 37 to 62 years;three of them had the history of hypertension, one of them accompanied with paroxysmal palpitation, while one case was asymptomatic. Computerized tomography and ultra-sonography showed single tumor in adrenal, the diameter was about 2.5、3.8、6.3、15cm respectively. Two cases showed positive results in 131Ⅰ-metaiodobenzylguanidine (MIBG) scintigraphy. One case showed positive result in 99Tcm-hydrazinonicotinamide-3trysinoctreotide (HTOC) scintigraphy. Twenty-four hours urinary catecholamine examination revealed the average of norepinephrine, epi-nephrine and dopamine were (196.1±92.2)nmol/24 h, (26.6±8.9)nmol/24 h, (1957.9±913.5) nmol/24 h respectively. Two cases were at a little elevated level and the others with normal. All cases were managed with α-adrenergic receptor blocker preoperatively for 2-4 weeks. Results Tumor re-section was performed in all 4 cases, and 3 cases by retroperitoneal laparoscopy, one by open surgery. All 4 cases were diagnosed as composite pheochromocytoma of adrenal (Pheochromocytoma-gangli-oneuroma) by pathology. All patients had no evidence of recurrence or metastasis during follow-up from 15 to 38 months. Two of three cases with hypertension reverted to normotensive. Conclusions Composite pheochromocytoma of adrenal is rare, difficult to be diagnosed preoperatively. Except mild in eatecholamine secretion, which clinical manifestations are similar to pheochromocytoma. Pheochro-mocytoma-ganglioneuroma is no apparent tendency for aggressive behaviors, the results of surgical management are favorable.