| Popis: |
Tumors of chromaffin cells derived from the embryonic neural crest are classified as pheochromocytomas when located in the medulla of the adrenal glands and paragangliomas when located externally to it. Bladder paragangliomas constitute less than 1% of all catecholamine-secreting neuroendocrine tumors. Usually, these tumors are functional and symptomatic, but in other cases, they can be silent. We present an interesting case of a patient with post-micturition catecholaminergic crises that went undiagnosed for several years until found to have a bladder paraganglioma. A 60-year-old male with a past medical history of TIA, HTN, CAD, and ischemic cardiomyopathy presented with post-micturition headaches since age 14. Despite work up, no etiology was found. Eventually, the patient was referred to mental health for what was thought to be somatic symptoms. In 2010, he was diagnosed with hypertension and started keeping daily blood pressure (BP) logs, which showed BP fluctuation as high as 260s/110s with high-volume micturition, yet normotensive at other times. This led to imaging and cystoscopy which revealed a 6-cm bladder lesion. Surgical resection was completed, and pathology showed paraganglioma with positive margins and lymphovascular invasion. He was lost to follow up until 2017 when he presented with hypertensive crisis. CT and octreotide scan showed numerous osseous lytic lesions concerning for metastases. Laboratory testing confirmed excess catecholamines. He was referred to oncology, and after consideration of several treatment options, the patient was started on Xgeva and Sandostatin LAR. However, he passed away shortly thereafter. Bladder paragangliomas are very rare and account for 6% of extra-adrenal pheochromocytomas. They occur more frequently in women than in men and clinically present mainly during the third decade of life. The patient typically suffers from hypertensive crises that may be accompanied by headache, palpitations, hot flushes, and sweating. These crises are mainly provoked by micturition, overdistention of the bladder, defecation, sexual activity, ejaculation, or bladder instrumentation. If there is high suspicion, biochemical and functional imaging workup should be performed. Surgery is the mainstay of the treatment and requires total excision. If diagnosed preoperatively, a partial cystectomy is preferred over trans-urethral resection as the majority of these tumors extend in the deep layers of the detrusor muscle. Because they are likely to recur and to metastasize, annual follow up with a measurement of plasma and urinary catecholamine levels and cystoscopy are essential. A functional imaging study (Ga-DOTATE scan) should be done to locate recurrence if symptoms reappear or catecholamine resurgence occurs. |
