关键词: 肾上腺肿瘤, 组合性嗜铬细胞瘤, 节细胞神经瘤

Abstract: Objective To investigate the clinical characteristics, management and prognosis of composite adrenal medullary tumor(pheochromocytoma-ganglioneuroma). Methods Eleven cases of composite adrenal medullary tumor were retrospectively analyzed on clinical materials. Summarize clinical manifestation, laboratory examinations, assistant examinations and pathological histology results, their follow-up data were got throμgh outpatient. Results Six cases were male,another five cases were female,aged 37 to 63 years.Ten of them had the history of hypertension,three of them accompanied with paroxysmal palpitation, while one case without any symptom.Twenty-four hours urinary catecholamine examination revealed that nine cases were at elevated level and the others were normal,the level of dopamine was elevated in six cases. Computerized tomography(CT) showed single tumor in adrenal gland, four cases in left, another seven cases in right. The diameter was 2.2 to 16.4cm, average 5.8cm. CT demonstrated that these tumours were heterogeneous and there was significant enhancement on postcontrast phase. Six cases had cystic components. Five of nine cases showed positive result in 99mTc-hydrazinonicotinamide-3 trypsin octreotide (HTOC) scintigraphy. Five cases all showed positive results in 131I-metaiodobenzylguanidine (MIBG) scintigraphy. Ten cases were managed with α-adrenergic receptor blocker preoperatively for 2 to 4 weeks. Tumor resection was performed in all 11 cases, and ten cases by retroperitoneal laparoscopy, one by open surgery. All 11 cases were diagnosed as composite pheochromocytoma of adrenal(Pheochromocytoma-ganglioneuroma) by pathology. All patients had no evidence of recurrence or metastasis during follow-up from 10 to 136 months.Nine of ten cases with hypertension reverted to normotensive. Conclusions Composite pheochromocytoma of adrenal is rare, difficult to be diagnosed preoperatively. Most cases were functional. Biopsy is the only accurate diagnostic method.Surgical resection is the best choice for treatment.Without malignant component, composite pheochromocytoma shows favourable prognosis.

Key words: adrenal gland neoplasms, composite pheochromocytoma, ganglioneuroma