Extra-adrenal pheochromocytoma: report of 34 cases / 中华泌尿外科杂志

ABSTRACT

Objective To review the diagnosis and treatment of extra-adrenal pheochromocytoma with a 34cases report. Methods Thirty-four cases of extra-adrenal pheochromocytoma were retrospectively analyzed. Hypertension was observed in 27 cases. Abdominal pain was seen in 10 patients and intermittent hematuria in 2 patients. Serum and urinary catecholamine and urinary VMA were measured in 34 cases. The level of serum or urinary catecholamine elevated in 20 cases and urine VMA elevated in 24 cases. Thirty-four cases had ultrasound examination,25 cases underwent CT scan and 6 cases underwent MER scan.Results Pheochromocytomas of 12 cases were located in the renal hilum, 2 in the lower pole of the left kidney, 1 in the posterior aspect of the inferior vena cava, 3 in the interaortocaval region, 2 in the anterior aspect of the abdominal aorta, 1 in the anterior of the right common iliac artery, 1 in the hilum of the liver, 1 in the posterior o{ the pancreas, 2 in the bladder wall, 1 in the posterior of the descending colon, and 8 cases of multifoci. Twenty-two cases of extraadrenal pheochromocytoma were benign and 12 cases were malignant. Thirty cases were followed up from 6 months to 13 years. Among 27 cases with hypertension, the blood pressure of 22 patients returned to normal and 5 cases were still hypertensive. Nine cases of malignant pheochromocytoma all had tumor recurrence or metastases at one year postoperatively. Six patients died during followed-up from 6 months to 3 years, including 3 cases died of cerebral hemorrhage and 3 cases of tumor metastases. Three cases got stable with 131Ⅰ-MIBG radiotheraphy.Conclusions The accurate detecting extra-adrenal pheochromocytoma is difficult. CT scan could be reliable in localizing the lesions. Surgical resection of the tumor could be the best therapy. Patients of malignant extra-adrenal pheochromocytoma may be treated with 131Ⅰ-MIBG after surgical therapy.