Phaeochromocytoma of the urinary bladder

Abstract

Phaeochromocytoma is a benign tumour which mostly occurs in the adrenal glands but can arise in extra-adrenal locations. We report a case in a Caucasian male who presented only with visible haematuria. Flexible cystoscopy revealed a mass on the left lateral wall, confirmed on a contrast-enhanced computed tomography scan of the urinary tract. The patient underwent a transurethral resection of bladder tumour with histological analysis demonstrating paraganglioma. Further reresection demonstrated a small nodule of recurrent disease in the lamina propria. Phaeochromocytoma of the urinary bladder is a rare condition which requires close investigation and follow-up due to its malignant potential. *Correspondence to: Sarmah PB, Department of Urology, City Hospital, Birmingham, UK, E-mail: piyushbsarmah@doctors.org.uk Received: April 23, 2020; Accepted: May 06, 2020; Published: May 11, 2020 Introduction Phaeochromocytoma commonly affects the adrenal gland but can have extra-adrenal locations due to distribution along the paraganglion system, including the urinary bladder. We report a case in a male patient without typical symptoms, and discuss the presentation, diagnosis and management of this condition. Clinical Practice Points A 56 year old male was seen in the Urology two week wait clinic with visible haematuria. He had undergone a flexible cystoscopy in his native country one month earlier and been informed that he had a bladder mass, however no biopsy was taken as he was taking Warfarin at the time for atrial fibrillation and he was advised to return to the UK. He had a further background history of Type II diabetes and hypertension, and was taking medications in the form of insulin, metformin, amlodipine, bisoprolol, ramipril and torasemide. He was a non-smoker and unemployed. On examination he was markedly obese. Abdominal examination revealed no tenderness or abdominal masses. There was a buried penis. Flexible cystoscopy was performed which demonstrated a large 3x4cm solid mass on the left lateral wall. Blood tests revealed impaired renal function but normal electrolytes (Table 1) and full blood count, and urine cytology and culture were normal. A computed tomography (CT) scan of the urinary tract with contrast was performed confirming the presence of a solid mass against the left lateral wall of the bladder with minor perivesical stranding (Figure 1), but normal upper urinary tracts. The patient underwent a transurethral resection of bladder tumour (TURBT) with the findings from flexible cystoscopy confirmed, and post-operatively had instillation of intravesical Mitomycin-C. Histology from the operative chippings included muscularis propria and found solid nests of cells diffusely arranged with a slightly packeted arrangement, separate by prominent vasculature. The tumour cells were immunoreactive for S100 and CD56, and overall appearances were consistent with an extra adrenal paraganglioma or phaeochromocytoma. A repeat TURBT and random bladder biopsy was performed two months later which confirmed a small nodule of recurrent extra adrenal phaeochromocytoma, located mainly in the lamina propria. A methyliodobenzylguanidine (MIBG) scintigram demonstrated no concurrent disease or residual disease in the wall of the bladder. The case was discussed at multi-disciplinary conference where the outcome was for a further re-resection in three months. The patient has subsequently undergone 2 years of surveillance but with no evidence of recurrence. Urea and electrolytes Sodium 135 mmol/L Potassium 4.9 mmol/L Urea 18.2 mmol/L Creatinine 165 umol/L Estimated glomerular filtration rate 45 mL/min/1.73m2 Table 1. Blood results on admission Figure 1. Axial section CT scan of the urinary tract demonstrating solid mass arising from left lateral wall of the urinary bladder Sarmah PB (2020) Phaeochromocytoma of the urinary bladder Volume 6: 2-2 Clin Res Trials, 2020 doi: 10.15761/CRT.1000304 Many treatment modalities exist for phaeochromocytoma of the bladder. Surgery in the form of either partial or radical cystectomy has been demonstrated as the preferred option in 70-80% of cases, most likely performed in order to achieve a greater degree of clearance with these submucosal tumours which have a greater malignant potential [6]. This has traditionally been performed via open repair but more recently laparoscopic and robot-assisted operations have been reported [8,9]. In this case the tumour was histologically non-invasive, and due to the patient’s obese state repeat TURBT was considered to be a safer option as he would have been high risk for major surgery. Regardless of the surgical intervention performed, lifelong follow-up and monitoring of disease recurrence is mandatory by measuring catecholamine levels owing to the potentially malignant nature of the condition [10].