Abdominal and pelvic extra-adrenal paraganglioma: a review of literature and a report on 7 cases.

摘要

INTRODUCTION: Extra-adrenal paraganglioma (pheochromocytoma) is a rare tumor. Herein we describe the clinical and pathological findings in patients with paragangliomas of the urinary bladder, seminal vesicle and retroperitoneum. METHODS: Between January 1994 and January 2001, extra-adrenal paragangliomas were diagnosed in 7 patients: 3 males and 4 females. The mean age of our patients was 32 +/- 15.9 years. We reviewed the clinical data. Urinary metanephrines and vanillyl mandelic acid and blood catecholamine levels were estimated in 4 cases. CT scan and/or MRI were used in the imaging of all cases. 123I-MIBG was used in only 1 patient, who harbored multiple tumors. All the patients but one underwent surgical treatment. RESULTS: The definitive diagnosis was made by histopathological examination of the removed tumors and was confirmed in all cases by the immunohistochemical stains of chromogranin A and S100 protein. There was metastasis in the pelvic lymph nodes in 1 patient. Follow-up ranged from 3 to 82 months (mean = 37.9 +/- 25.8). The catecholamine level was elevated in 3 patients under basal conditions and during endoscopic resection of the tumor in a fourth patient. In all cases, the catecholamine level was normalized after surgery. There was no recurrence or metastasis in any case following surgery. CONCLUSION: Pre-operative diagnosis of nonfunctioning bladder paraganglioma is difficult, but the tumors should be suspected in patients who have hypertension, hematuria or mass effects due to the tumor growth in the pelvis and/or retroperitoneum. Six of the seven cases reported here were found in the usual locations: 3 in the urinary bladder, 2 in the renal hilum and 1 in the organ of Zuckerkandl. One patient had multiple tumors, including a paraganglioma of the seminal vesicles. Resection is the treatment of choice, and in the case of urinary bladder paraganglioma should include total cystectomy. In patients with unresectable multiple tumors, medical therapy may be used to control hypertension.