Diagnosis and treatment of ectopic pheochromocytoma

Objective To summarize clinical characteristics of ectopie pheochromocytoma in order to improve the diagnosis and treatment of this disease.Methods Clinical data of 36 cases of pathologically proved ectopic pheochromocytoma treated in Tianjin Medical University General Hospital from 1990 to 2010 were retrospectively analyzed.Hypertension was observed in 28 cases.5 cases had hypertension at the end of urination. 1 case was proved to have ectopic pheochromocytoma by physical examination.Abdominal pain occulted in 2 patients.Urinary VMA was measured in all the 36 cases,and 32 esges had an elevation of VMA(88.9%).The lesions were localized mainly by abdominal ultrosonggraphy,CT,MRI and 131 I-MIBG.All cages underwent operation,including 4 laparoscopic surgery.Results Of the 36 patients with ectopic pheochromocytoma,34 cases had sinsle tumor,and 2 cases had multiple tumors.The diameter of the lesions was from 3.4 cm to 18.2 cm in size.Postoperative pathological examination showed 27 cases of ectopic pheochromocytoma were benign and 9 cases were malignant.Conclusions VMA level is an important marker to detect ectopic pheochromocytoma.Tumor localization is difficult.131 I-MIBG is of great value for localization of the lesion.Meticulous preoperative preparation is extremely important.Surgical resection of the tumor is the best therapy.Laparoscopic surgery can be the new choice for treatment of ectopic pheochromocytoma.Patients with malignant ectopic pheochromocytoma may be treated with 131 I-MIBG after surgery. Key words: Ectopie pheochromocytoma