We describe a case of a 63-year-old Chinese female presenting with a year long history of headaches, palpitations and lower abdominal fullness on a background of hypertension. Initial investigations were suggestive of malignancy with elevated tumour markers; CA125 58 U/ml (N: <30) and CA19.9 126 U/ml (N: <39). A pelvic ultrasound revealed a hypervascular bladder wall mass measuring 22 x 15 x 16mm, concerning for a transitional cell carcinoma. MRI pelvis further characterised the lesion to have extended outside the bladder wall and demonstrated diffusion restriction.
An elective laparoscopic total abdominal hysterectomy, bilateral salpingo-oophorectomy, cystoscopy and TURBT was organised. At the time of the biopsy the blood pressure spiked to 210/120mmHg from 120/80mmHg which was managed with intravenous hydralazine. Serum metanephrines and normetanephrines were ordered and were elevated at 640pmol/L (N: <447) and 1920pmol/L (N: <1160) respectively. Histopathology confirmed the diagnosis of a bladder paraganglioma. A PET Gallium-68 DOTATE scan found no significant DOTATE activity within the bladder wall or any metastatic disease. The patient was pre-operatively stabilised on phenoxybenzamine and propranolol commenced three days later. A cystoscopy and robotic assisted partial cystectomy was performed successfully 10 days later with minimal handling of the mass. The patient had an excellent post operative recovery with remission of her hypertension and palpitations.
Bladder paragangliomas are extremely rare neuroendocrine tumours accounting for less than 0.06% of bladder tumours and less than 1% of all phaeochromocytomas and paragangliomas (1). The most common symptoms include painless haematuria, hypertension and micturition attacks (2). Due to the rarity of these tumours diagnosis is often difficult. CT and MRI may play a role in assisting with diagnosis preoperatively such that a catecholamine crises may be avoided intraoperatively. On MRI these tumours have T2 hyperintensity, slight T1 hyperintensity compared to muscle and marked restricted diffusion on DWI (3).