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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2012  |  Volume : 23  |  Issue : 4  |  Page : 813-816
Pheochromocytoma of the urinary bladder: A rare cause of severe hypertension


1 Department of Surgical Oncology, Gujarat Cancer and Research Institute, Ahmedabad, India
2 Department of Surgical Oncology, Kasturba Medical College, Manipal, India

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Date of Web Publication9-Jul-2012
 

   Abstract 

Paraganglioma of the urinary bladder is a rare entity, and accounts for less than 0.5% of all bladder tumors. In the genitourinary tract, the urinary bladder is the most common site for paragangliomas. These tumors commonly present with hematuria and intermittent hyper­tension during micturition along with generalized symptoms due to raised catecholamines. Surgical removal is the treatment of choice, with most of the patients requiring at least a partial cystectomy. We herewith report a 38-year-old lady who presented with a two month history of pain abdomen, burning micturition, headache and uncontrolled hypertension. Detailed investi­gations revealed a pheochromocytoma of the urinary bladder, which was treated with partial cystectomy, following which her blood pressure stabilized.

How to cite this article:
Hanji AM, Rohan VS, Patel JJ, Tankshali RA. Pheochromocytoma of the urinary bladder: A rare cause of severe hypertension. Saudi J Kidney Dis Transpl 2012;23:813-6

How to cite this URL:
Hanji AM, Rohan VS, Patel JJ, Tankshali RA. Pheochromocytoma of the urinary bladder: A rare cause of severe hypertension. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2014 Oct 25];23:813-6. Available from: http://www.sjkdt.org/text.asp?2012/23/4/813/98167

   Introduction Top


Paraganglioma of the urinary bladder is a rare entity and accounts for less than 0.5% of all bladder tumors. Paragangliomas have been re­ported to arise from the orbit, ear, nose, larynx, carotid area, mediastinum, duodenum and the genitourinary tract. [1] In the genitourinary tract, the urinary bladder is the most common site for paragangliomas (79.2%), followed by the ure­thra (12.7%), pelvis (4.9%) and ureter (3.2%). [2]


   Case Report Top


A 38-year-old lady presented with a two months history of pain abdomen, burning mic­turition and headache. On examination, she had hypertension of 200/110 mmHg. There was no family history of hypertension. The patient was on multiple anti-hypertensive drugs, despite which her blood pressure was not adequately controlled. Urinary vanillylmandelic acid was tested, which showed a value of 26 mg/g (nor­mal: 2-8 mg/g). Echocardiography showed concentric left ventricular hypertrophy. Com­puterized tomographic (CT) scan of the abdo­men and pelvis was performed, which showed a 40 mm × 45 mm × 55 mm lobulated hypo-dense lesion with internal cystic areas situated at the right postero-lateral wall of the urinary bladder, involving the right vesicoureteric junc­tion and the lower end of the right ureter, leading to right hydrouretronephrosis. Intense early arterial phase enhancement was seen [Figure 1]. On the basis of clinical and ima­ging findings, a diagnosis of extra-adrenal pheopheochromocytoma of the urinary bladder was considered. Cystoscopic findings further con­firmed our diagnosis. Pre-operatively, her hy­pertension was controlled with an alfa blocker (prazosin). Partial cystectomy and excision of the lower end of the right ureter along with re­implantation of the right ureter was success­fully performed. Her blood pressure was well controlled intra-operatively.
Figure 1: Computerized tomography scan of the pelvis showing a 40 mm × 45 mm × 55 mm lobulated hypodense lesion with internal cystic areas situated at the right postero-lateral wall of the urinary bladder, involving right vesicoureteric junction.

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Gross examination of the tumor revealed a 5 cm × 4.2 cm × 3.4 cm growth, the cut-surface of which was yellowish and firm in consis­tency. Microscopically, the tumor showed growth pattern located in the sub-mucosal region with infiltrates extending from the lamina propria up to the serosa. The peri-vesical fat was free. The cells presented a pale to pink granular cytoplasm, with small nucleus and a low N:C ratio [Figure 2]. Immunohistochemical analy­sis revealed a positive reaction to vimentin, chromogranin, neuron-specific enolase and synaptophysin, compatible with a diagnosis of pheochromocytoma.
Figure 2: Microscopic examination of the resected tumor showing cells with a pale to pink granular cytoplasm with small nucleus (H&E 10×).

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Post-operatively, her blood pressure stabilized. She remains asymptomatic and is on regular follow-up for the past 15 months with well-controlled blood pressure.


   Discussion Top


Pheochromocytomas of the urinary bladder arise from the chromaffin tissues of the sym­pathetic nervous system within all layers of the bladder wall. It is a rare neoplasm, accounting for less than 0.5% of all bladder tumors and less than 1% of all pheochromocytomas. [3]

Extra-adrenal pheochromocytomas are found in 10% of adults and up to 30% in children suffering from pheochromocytomas. [3] Most of the pheochromocytomas of the urinary bladder occur in young and middle-aged adults, and are more common in women. Paragangliomas usually have a benign clinical course, with only 10% being malignant. The cytologic fea­tures of benign and malignant tumors overlap and, thus, there are no reliable features of malignancy. [4]

These tumors commonly present with hematuria and intermittent hypertension during mic­turition along with generalized symptoms due to raised catecholamines such as headache, blurred vision, palpitations and flushing. The classical triad of episodic hypertension, persis­tent hematuria and post-micturition syncope is virtually diagnostic, but is very rare. [1] Our patient presented with hypertension and head­ache but with no hematuria. Commonly, urinary bladder pheochromocytomas are hormonally active with elevated catecholamine metabolites. [3] Imaging studies are useful in locali­zing the tumor. On ultrasonography (USG), pheochromocytomas appear as sharply demar­cated soft tissue masses that may be purely solid or may contain foci of hemorrhage and necrosis that appear cystic. [5] CT scan is more sensitive than USG in detecting adrenal (94%) and extra-adrenal pheochromocytomas. Mag­netic resonance imaging (MRI) is more sen­sitive than CT scan for detecting small extra-adrenal pheochromocytomas. I 131 meta-iodobenzyl-guanidine (MIBG) scan serves as a complementary functional diagnostic tool, but its use is limited because of its expense and restricted availability. [6]

Cystoscopic examination aids in delineating the exact location of the lesion with regard to the depth of invasion and involvement of the ureters. On cystoscopy, pheochromocytomas appear as granulated and lobulated lesions, with or without ulceration. [7] Role of biopsy for confirmation is debatable. [2]

Treatment options include trans-urethral re­section, laparoscopic or open cystectomy (par­tial or radical) with the aim of complete local excision of the tumor. [8] Surgical removal is the treatment of choice. Because the sympathetic plexus of the bladder is scattered between all layers of the bladder, at least a partial cystectomy will be required. [9] This can be done either by open surgery [10],[11] or through laparoscopy. [12] In patients with functioning tumors, it is recommended to be very careful in the pre­and intra-operative period in order to prevent hypertensive crises.

Radiotherapy is only indicated in unresectable lesions, positive margins or patients unfit for surgery. [9] The role of chemotherapy is con­troversial, and is given in advanced cases and in metastatic disease. Most commonly used drugs are cyclophosphamide, vincristine and dacarbazine (CVD). [13],[14],[15]

Prognosis depends on the presence of metas­tases, size of the lesion and familial endocrinopathy. [10] Patients with pheochromocytoma need prolonged follow-up to detect local recu­rrences or metastases that have been described 20 and 40 years after treatment. [16] To summarize, a high index of suspicion is necessary to diagnose pheochromocytoma of the urinary bladder as it is a rare tumor. In patients with uncontrolled hypertension or hypertension fluctuating with micturition, a diagnosis of pheochromocytoma should be considered. Suspected cases should be tho­roughly investigated by imaging studies such as CT and MRI scans before venturing to surgery.

 
   References Top

1.Goyal S, Puri T. Skeletal metastases from recurrent paraganglioma of the urinary bladder. Indian J Urol 2005;21:122-4.  Back to cited text no. 1
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2.Dahm P, Gschwend JE. Malignant non-urotelial neoplasms of the urinary bladder: a review. Eur Urol 2003;44:672-81.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Naqiyah I, Rohaizak M. Phaeochromocytoma of the urinary bladder. Singapore Med J 2005; 46:345  Back to cited text no. 3
    
4.Siatelis A, Konstantinidis C, Volanis D, Leontara V, Thoma-Tsagli E, Delakas D. Pheochromo-cytoma of the urinary bladder: report of 2 cases and review of literature. Minerva Urol Nefrol 2008;60:137-40.  Back to cited text no. 4
[PUBMED]    
5.Crecelius SA, Bellah R. Pheochromocytoma of the bladder in an adolescent: Sonographic and MR imaging findings. Am J Roentgenol 1995; 165:101-3.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Gittes RF, Mahoney EM. Pheochromocytoma. Urol Clin North Am 1997;4:239-52.  Back to cited text no. 6
    
7.Doran F, Varinli S, Bayazit Y, Bal N, Ozdemir S. Pheochromocytoma of the urinary bladder. APMIS 2002;110:733-6.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Heinrich E, Gattenloehner S, Mueller-Hermelink HK, Michel MS, Schoen G. Paraganglioma of Urinary Bladder. Urol J 2008;5:57-9.  Back to cited text no. 8
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9.Gustavo CG. Renato Mazon Lima Verde Leal. Urinary Bladder Paraganglioma. Appl Cancer Res 2006;26:76-9.  Back to cited text no. 9
    
10.Bonacrzu Kazzi G. Asymptomatic bladder phaeochromocytoma in a 7-year-old boy. J Paediatr Child Health 2001;37:600-2.  Back to cited text no. 10
    
11.Nakatani T, Hayama T, Uchida J, Nakamura K, Takemoto Y, Sugimura K. Diagnostic locali­zation of extra-adrenal pheochromocytoma: Comparison of (123)I-MIBG imaging and (131)I-MIBG imaging. Oncol Reports 2002;9: 1225-7.  Back to cited text no. 11
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12.Kozlowski PM, Mihm F, Winfield HN. Laparoscopic management of bladder pheochromocytoma. Urology 2001;57:365.  Back to cited text no. 12
[PUBMED]  [FULLTEXT]  
13.Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocytoma. J Urol 1992;147: 1-10.  Back to cited text no. 13
[PUBMED]    
14.Zwahlen D, Fishman PN, Honey J, Milosevic M, Tannock I. Malignant pheochromocytoma of the urinary bladder. Can J Urol 2007;14:3455-7.  Back to cited text no. 14
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15.Buonomo V, Ruggiero A. Malignant retro-peritoneal paraganglioma: Case report and review of the literature. Pediatric Hematol Oncol 2004;21:731-8.  Back to cited text no. 15
    
16.Mrabet N, Bellil K, Bellil S, et al. Para-ganglioma of urinary bladder: Report of two cases Tunis Med 2007;85:163-5.  Back to cited text no. 16
    

Top
Correspondence Address:
Vinayak S Rohan
Department of Surgical Oncology, Kasturba Medical College, Manipal
India
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DOI: 10.4103/1319-2442.98167

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