Retrospective analysis of the diagnosis and treatment of urinary bladder paragangliomas

Abstract

Objective To discuss the clinical and pathological manifestations of paragangliomas of the urinary bladder (PUB) and improve the preoperative diagnosis and surgical treatment of PUB.Methods Clinical data of 11 patients treated for PUB from June 1985 to March 2010 were analyzed.All cases had mild-paroxysmal hypertension, palpitation, sweating, and 9 cases occasionally showed headache and micturition syncope during straining urination. Twenty-four hour urine catecholamine (CA) levels were estimated in all cases. B-ultrasound and CT and/or MRI scanning were used in the imaging of all cases. Nailfola microcirculation inspection was carried out in 5 cases. Scintigraphy 131I-MIBG in 4 patients was positive expression, 7 of 111 In-DTPA-Octreotide scintigraphy and I of PETCT were also positive expression. UICC bladder tumor classification was T1 (1 case), T2 (5 cases), T3(4 cases), T4 (1 case). Results All the patients underwent partial cystectomy, laparoscopy or TURBT. One patient received 131 I-MIBG therapy. Histopathological diagnosis was confirmed by HE staining in all of the removed tumors. The tumors consisted of discrete aggregates of zellballen cells separated by a network of vascular channels. Follow-up ranged from 3 to 291 months (mean, 45 months). There were 3 recurrences following surgery. One patient died after developing pelvic lymph nodes, liver and colon metastasis. Conclusions PUB should be suspected in patients below 40 years of age if the clinical manifestations of typical tetrad symptoms: headache and micturition syncope,sweating, palpitation and hematuria are present. Advanced classification, multifocal tumors and CgA (+) are risks of recurrence and metastasis. In those patients with unresectable multiple or recurrent tumors, chemotherapy and 131I-MIBG therapy may be helpful for controling hypertension and delaying progress. Key words: Paraganglioma; Catecholamine; Hypertension; Micturition syncope; Urinary bladder neoplasms