排尿後，就頭痛?- 副神經節瘤案例報告與文獻回顧

黃英哲¹ 、劉惠瑛¹

¹高雄長庚紀念醫院外科部泌尿科

Headache after urination: A case report and review of the sympathetic paraganglioma

Ying-Che Huang¹、Hui-Ying Liu¹

¹Divisions of Urology, Department of Surgery, Kaohsiung Chang Gung Memorial Hospital

Case description: A 11-year-old girl, with no significant medical history, presented to our outpatient department with a chief complaint of headache after urination for more than 10 years. A computed tomography (CT) of the brain and an electroencephalogram (EEG) were ordered, which were negative for significant findings. During admission, hypertension was noted and the nephrologist was consulted for a workup. Results were as follows: aldosterone 10 ng/dL (Lab ref. 4.9-17.5 ng/dL), renin 28.3 ng/L (Lab ref. 3.2-32.6 ng/L), cortisol 8.76 ug/dL (Lab ref. 2.5-12 ug/dL), ACTH 50.6 pg/ml (Lab ref. 7.2-63.3 pg/ml), metanephrine <0.18 nmol/L (Lab ref. <0.5 nmol/L), normetanephrine 7.69 nmol/L (Lab ref. <0.9 nmol/L), urine vanillylmandelic acid (VMA) 15.1 mg/day (Lab ref. 1.9-9.8 mg/day) and catecholamine- norepinephrine >988 ug/day (Lab ref. <80 ug/day). The complete blood count and comprehensive metabolic panel were within normal range. CT of the abdomen found avidly enhanced nodular masses in the left pelvis, abutting to left urinary bladder wall and rectum. MRI showed a left pelvic lobulated enhancing mass (4.5x3.2x3.6 cm) between the urinary bladder and obturator vessel. Under the clinical diagnosis of sympathetic paraganglioma (PG), a pre-operation alpha-blocker was added. The patient was then scheduled for robotic tumor excision and partial cystectomy. On histopathological examination, there was one tissue fragment, measuring 4.4 x 3.7 x 3.0 cm. It was a yellowish-tan and solid tumor. No hemorrhage or necrosis is noted. Microscopically, the tumor invaded to muscularis of the propria of the urinary bladder, but the margin is uninvolved. In immunohistochemistry, it was positive for synaptophysin, chromogranin A (focal), and S100 (focal weakly, sustentacular cells). The result is consistent with PG. The postoperative course was uneventful. Anti-hypertensive medications were tapered and the patient received follow-up with plans to re-image six months later in our Urology outpatient department.

Discussion: PGs develop from the chromaffin cells of the sympathetic ganglia and are catecholamine-secreting neuroendocrine tumors. Different from pheochromocytomas growing inside the adrenal gland, PGs are extra-adrenal tumors. The symptoms of PGs can vary greatly, but the most common triad are episodic headache, sweating, and tachycardia¹. Initial evaluation includes biomedical testing and image study. Biochemical diagnosis involves the measurement of 24h urine catecholamines, serum metanephrine, and urinary VMA^2,3. Once the presence of excess catecholamines is detected, imaging studies including CT and MRI can be used to characterize and localization.  CT will show avid enhancement following the administration of contrast media. MRI offers detailed pictures of adjacent vascular structures². Currently, surgery is the primary treatment for PGs. To prevent intraoperative hypertensive crisis, appropriate administration of anti-hypertensive medications including α-blocking and β-blocking agents is crucial before surgery. Histologically, PGs are composed of chief epithelioid cells arranged in a nested fashion; sustentacular cells are peripheral to the chief cell nests. Immunohistochemical staining is typically positive for neuron-specific enolase, synaptophysin, and/or chromogranin for chief cells and may be positive for S100 for sustentacular cells^4,5. However, previous literature did not find histological criteria for malignant PGs.

Conclusions: In this manuscript, we presented a rare case of sympathetic paraganglioma in an 11-year-old girl. She presented with headache after urination for more than 10 years and the subsequent study showed the presence of a pelvic mass abutting the bladder. The mass was surgically removed and consistent with PG. Although most of the PGs behave benignly, malignant transformation does occur in a few cases. Further outpatient follow-up is needed for those patients.