肾上腺组合性嗜铬细胞瘤四例报告及文献复习

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.

Composite pheochromoeytoma of adrenal: report of 4 cases