Urinary Bladder Paraganglioma presenting as Micturition-Induced Palpitations, Dyspnea, and Angina

Patient: Female, 45 Final Diagnosis: Urinary bladder paraganglioma Symptoms: Angina pectoris • dyspnea • palpitations Medication: Phenoxybenzamine • Propanolol Clinical Procedure: Open partial cystectomy Specialty: Endocrinology and Metabolic OBJECTIVE: Rare disease BACKGROUND: Sympathetic urinary bladder paragangliomas are rare catecholamine-secreting neuroendocrine tumors arising from neural crest cells. They are uncommon urinary bladder neoplasms. Symptoms classically include micturition-related or unrelated palpitations and syncope with hypertension, headaches, diaphoresis, and hematuria. Other than being attributable to vasovagal reactions, micturition-induced cardiovascular symptoms should prompt a search for catecholamine-secreting tumors such as a urinary bladder paraganglioma, as in this case. CASE REPORT: A 45-year-old asthmatic African-American female presented with episodic hematuria that began 4 years ago and episodes of micturition-induced palpitations, dyspnea, substernal tightness, sweating, and throbbing headaches. Computed tomography with contrast revealed an enhancing mass along the anterior urinary bladder wall, measuring 2.4×3.5 cm. On Positron emission Tomography with [18F] fluorodeoxyglucose integrated with computed tomography (18F-FDG PET/CT), the urinary bladder mass was 18F-FDG avid. Serum normetanephrine and supine plasma norepinephrine were significantly elevated and there was mild elevation of supine plasma epinephrine. Transurethral resection of the bladder mass revealed a neoplasm with microscopic features and immunohistochemical profile positive for synaptophysin and chromogranin, with negative screening cytokeratin AE1/AE3, suggesting a paraganglioma. Following resection of the paraganglioma, there was complete resolution of micturition-induced cardiovascular symptoms on long-term follow-up. CONCLUSIONS: Micturition-related cardiovascular symptoms are commonly attributed to vasovagal reactions. However, urinary bladder pathologies must be ruled out as a cause, as in this rare case of a urinary bladder paraganglioma exhibiting catecholaminergic symptoms.