Paraganglioma of the Urinary Bladder: A Case Report and Review of Literature

Case Report

Austin J Urol. 2015;2(3): 1028.

Paraganglioma of the Urinary Bladder: A Case Report and Review of Literature

Sriram Krishnamoorthy*, Leena Dennis Joseph, Susruthan M, Kanimozhi G, Sunil Shroff

Department of Urology, Sri Ramachandra Medical College, India

*Corresponding author: Sriram Krishnamoorthy, Additional Professor, Department of Urology, Sri Ramachandra. Medical College & RI, Chennai, India

Received: March 21, 2015; Accepted: March 23, 2015; Published: July 30, 2015


Paraganglioma of the urinary bladder is a rare tumour with characteristic histopathological and immunehistochemical features. It is difficult to differentiate the tumour as benign or malignant based on the histological findings alone. Recognition of this tumour is important for the proper management of these patients, as these patients may or may not be having bothersome symptoms.

Extra adrenal pheochromocytomas are known as paragangliomas. Majority of these Extra adrenal tumours occur intraabdominally along the sympathetic chain. These tumours of the sympathetic nervous tissue may be non-functional or functional and when they secrete catecholamine, they cause paroxysmal hypertension, palpitation, and micturition syncope.

The current report presents a case of a 36 year old male, who was suffering from recurrent dysuria and vague supra pubic discomfort for the past six months. Clinical evaluation and imaging were normal. Cystoscopy revealed a small sessile polyp over the dome of the bladder. Histopathology showed the characteristic ‘zellballen’ pattern which was confirmed by immunohistochemical staining. The purpose of this case report is to highlight the rarity of the tumour, the various unusual modes of presentation and to stress on the need to forewarn the surgeons and the anaesthetists of the possible intra-operative hypertensive crisis during resection of such tumours.

Keywords: Paraganglioma; urinary bladder; synaptophysin


Pheochromocytomas are tumours of the chromaffin cells, derived from the embryonic neural crest. They are most commonly located in the adrenal medulla. However 10% of these tumours are reported at Extra adrenal locations [1]. Such Extra adrenal pheochromocytomas are known as paragangliomas. Majority of these Extra adrenal tumours occur intra abdominally along the sympathetic chain. Primary paragangliomas of the urinary bladder are very rare, accounting for less than 0.05% of all bladder neoplasms and less than 1% of all pheochromocytomas[2]. Paraganglioma of the urinary bladder is a rare tumour that originates from the embryonic rests of chromaffin tissue of the sympathetic nervous system associated with the urinary bladder wall. Nearly 80% of the paragangliomas of the genito urinary tract arise from the urinary bladder, followed by a decreasing order of frequency involving the urethra, renal pelvis and the ureters [3].

These patients usually present with a symptom complex of hypertension, headache, sweating and palpitation associated with micturition. These are attributed to the catecholamine release from the tumour.

Case Report

We report a case of a 36 year old male patient, who presented with complains of recurrent mild dysuria and supra pubic discomfort for the past six months. He also complained of burning micturition. There was no history of hematuria, pyuria or lithuria. The patient did not complain of fever, chills nausea or vomiting. He is a known hypertensive for the past three years, on regular medication. There was no history suggesting a paraganglioma of the bladder like micturition syncope, headache, sweating or palpitation. He is non alcoholic, non smoker with regular bowel habits. On physical examination, his general condition was good. All the laboratory parameters were within normal limits. Urine examination was normal and urine culture was sterile. Ultrasound of the bladder and kidneys were normal and uroflow showed a satisfactory voiding pattern with insignificant residual urine.

In view of unexplained lower urinary tract symptoms, cystoscopy was done which showed a small sessile polyp measuring approximately 0.5 cms over the dome of the bladder. A clinical suspicion of urothelial lesion was suggested. The tumour was resected and sent for histopathological examination as multiple tiny fragments, which were all embedded.

On light microscopy, the tumour cells were seen as well circumscribed nodules with a characteristic ‘zellballen’ pattern (Figure 1). The individual tumour cells were polygonal with a granular cytoplasm. There was a delicate fibro vascular stroma surrounding the tumour cell clusters (Figure 2). A provisional diagnosis of paraganglioma was given with an advice for immunohistochemical confirmation. Immunohistochemistry stains were positive for synaptophysin, vimentin and chromogranin (Figure 3). There was no invasion into the underlying muscle. There was no evidence of necrosis or abnormal mitosis. As the tumour has been completely resected, the patient was advised to come for a regular follow up.