Podium 01
膀胱副神經節瘤合併術中高血壓：病例報告及文獻回顧
張廷旭1
Bladder paraganglioma with Intraoperative Hypertensive Crisis: A Case report and literature review
Jeong Uk Jang1
Department of Urology1, Ditmanson Medical Foundation Chiayi Christian Hospital, Chia-Yi, Taiwan
Pheochromocytomas are tumors arising from chromaffin cells of the adrenal medulla, while approximately 10% occur in extra-adrenal locations and are termed paragangliomas. Paragangliomas of the urinary bladder are extremely rare, accounting for less than 0.05% of all bladder tumors and approximately 6–10% of extra-adrenal pheochromocytomas. Clinical manifestations are variable and may include hypertension, headache, palpitations, and hematuria. Functional tumors may cause catecholamine-related symptoms, especially during micturition. Here, we present a case of bladder paraganglioma initially misdiagnosed as urothelial carcinoma, with intraoperative hypertensive crisis during surgical resection.
A 59-year-old man with a history of hypertension and hyperlipidemia under medical treatment presented with intermittent painless hematuria for one month. Physical examination was unremarkable. Urinalysis revealed numerous red blood cells, and urine culture was negative. Contrast-enhanced abdominal computed tomography (CT) demonstrated a 1.4 cm enhancing mass located on the right inferior wall of the urinary bladder. Cystoscopic examination revealed a submucosal tumor over the right lateral wall. With a presumptive diagnosis of urothelial carcinoma, the patient underwent transurethral resection of bladder tumor (TURBT). During the procedure, a sudden elevation in blood pressure exceeding 190 mmHg was noted, raising suspicion of a catecholamine-secreting tumor. The operation was completed without further complications, and the postoperative course was uneventful.
Histopathological examination demonstrated a characteristic Zellballen pattern. Immunohistochemical staining showed strong positivity for synaptophysin and chromogranin, confirming the diagnosis of bladder paraganglioma. The tumor was classified as pT2 based on invasion into the muscularis propria.
Bladder paraganglioma is a rare neuroendocrine tumor arising from chromaffin cells within the bladder wall. It accounts for approximately 0.05–0.06% of all bladder tumors and about 6% of extra-adrenal pheochromocytomas. It is more commonly reported in women and typically occurs in patients aged 40–50 years. The urinary bladder is the most common site of genitourinary paragangliomas, comprising approximately 79% of cases. Other sites include the urethra, renal pelvis, and ureter. Clinical presentation varies widely. Up to 80% of patients may exhibit functional symptoms related to catecholamine secretion, including hypertension, palpitations, headache, and micturition syncope. However, nonfunctional tumors may present solely with painless hematuria, as in our case, making preoperative diagnosis challenging. Cystoscopic findings typically reveal a submucosal mass, which can be misdiagnosed as urothelial carcinoma. Intraoperative hypertensive crisis should raise suspicion for paraganglioma. Surgical resection remains the mainstay of treatment. Partial cystectomy is generally preferred over TURBT due to the frequent involvement of the detrusor muscle. The reported local recurrence rate is approximately 10–20%, and 10–15% of tumors may exhibit malignant behavior with metastasis. Given the potential for recurrence and metastasis, long-term follow-up is recommended.
Bladder paraganglioma is a rare entity that may mimic urothelial carcinoma, particularly when presenting as a submucosal bladder mass. This case highlights the importance of considering paraganglioma in the differential diagnosis of bladder tumors, especially when unexplained intraoperative hypertension occurs. Early recognition is crucial to prevent perioperative complications associated with catecholamine release. Complete surgical excision remains the cornerstone of treatment, and long-term surveillance is essential due to the risk of recurrence and potential malignant behavior.