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Year : 2005  |  Volume : 21  |  Issue : 2  |  Page : 122-124

Skeletal metastases from recurrent paraganglioma of the urinary bladder.

1 Department of Radiotherapy & Oncology,All India Institute of Medical Sciences,New Delhi - 110029, India
2 Department of Radiodiagnosis,All India Institute of Medical Sciences,New Delhi - 110029, India
3 Department of Pathology,All India Institute of Medical Sciences,New Delhi - 110029, India

Correspondence Address:
Shikha Goyal
Department of Radiotherapy & Oncology,All India Institute of Medical Sciences,New Delhi - 110029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-1591.19637

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Paragangliomas arising from the urinary bladder are extremely rare. Most of them are benign, and are curable with surgical resection alone, but about 10% are malignant and may metastasise to regional nodes or distant sites. We present the case of a 45 year male patient who had a recurrent paraganglioma of the urinary bladder with vertebral metastases. The patient was managed using a combination of palliative chemotherapy with carboplatin, etoposide and zoledronate and radiotherapy to the spine. However, the patient's general condition rapidly deteriorated while on systemic treatment. This case demonstrates the unusually aggressive course that this disease may occasionally take.

Keywords: Paraganglioma; Urinary bladder; Skeletal metastases

How to cite this article:
Goyal S, Puri T, Gunabushanam G, Das AK, Julka PK. Skeletal metastases from recurrent paraganglioma of the urinary bladder. Indian J Urol 2005;21:122-4

How to cite this URL:
Goyal S, Puri T, Gunabushanam G, Das AK, Julka PK. Skeletal metastases from recurrent paraganglioma of the urinary bladder. Indian J Urol [serial online] 2005 [cited 2022 Dec 8];21:122-4. Available from:

   Introduction Top

Paragangliomas or extraadrenal pheochromocytomas arise from the chromaffin tissue in the sympathetic ganglia, and are derived from the embryonic neural crest cells. They account for 10% of all pheochromocytomas. Paragangliomas of the urinary bladder represent less than 1% of bladder tumours and 6% of all paragangliomas.[1] Of these, only about 10% show malignant behaviour in the form of local recurrence or distant metastases.[2] Vertebral metastases are exceedingly rare, with few isolated case reports in literature.

We present the case of a recurrent paraganglioma of the urinary bladder with multiple vertebral metastases.

   Case report Top

A 45 year man presented to our department with a recurrent paraganglioma of urinary bladder. The patient never had manifestations of catecholamine hypersecretion, such as diaphoresis, hypertension, or anxiety. MIBG scan done at the time of initial diagnosis failed to reveal uptake of radiotracer in the tumour. He had been managed surgically by total cystectomy and ileal conduit for his primary tumour. He was followed with serial abdominal CT scanning for four years till October 2004, when a local pelvic recurrence with presacral and pelvic soft tissue deposits were detected. Subsequently he underwent an exploratory laparotomy and tumour excision. Histopathology of the postoperative specimen [Figure - 1]A and B showed a paraganglioma.

The patient was started on adjuvant pelvic radiotherapy on account of recurrent disease. A dose of 45 Gray in 25 fractions over 5 weeks was planned to whole pelvis. He was also given symptomatic treatment for pelvic pain. During the course of radiotherapy, he developed backache of progressively increasing severity with associated lower limb weakness. On examination, power in the lower limbs deteriorated from 4/5 to 2/5 over a period of 3 days. MRI spine showed collapse of the C7 vertebral body with preservation of the intervertebral disc spaces, and cord compression at the same level [Figure - 2].There was an alteration in the signal intensity at the D10 and D11 vertebral levels. The imaging findings were diagnostic of spinal metastases.

Pelvic radiotherapy was discontinued and the patient was given palliative radiotherapy of 8 Gray in a single fraction to C7 and D10-D11 vertebrae, as per institutional protocol for vertebral metastases. Pain was managed with NSAIDs and opioid analgesics. He was subsequently started on palliative chemotherapy with carboplatin (AUC 5 iv D1) and etoposide (100 mg/m[2] iv D1, D2, D3) along with zoledronate (4 mg iv D1) as 3-weekly cycles. His general condition deteriorated over the course of systemic treatment and there was no response to chemotherapy and bisphosphonates in terms of analgesic requirement or neurologic deficit. After completing six cycles, further active management was stopped and he was referred to a hospice for supportive care.

   Discussion Top

During embryogenesis, primitive neural crest cells migrate to various locations in the body and differentiate into chief and sustentacular cells, forming the neuroendocrine or paraganglion system of the autonomic nervous system. Tumours of these cells originating from the adrenal medulla are termed the pheochromocytomas, while extraadrenal tumours are called paragangliomas. Paragangliomas have been reported to arise from the orbit, nose, ear, larynx, carotid area, mediastinum, retroperitoneum, urinary bladder, and duodenum. These tumours cannot be classified as benign or malignant based on any morphological criteria, with only the presence of metastatic disease serving as a proof of malignant behaviour.[3] Most paragangliomas follow a benign clinical course, only 10% being malignant. Local recurrences or metastases have been reported to occur as late as 30 years after surgery, with regional lymph nodes, lungs and bones being the commonest sites.

Paraganglioma of the urinary bladder is a rare tumour, comprising 0.06% of all bladder tumours and 6% of paragangliomas. The classic triad of episodic hypertension, persistent hematuria and post-micturition syncope is virtually diagnostic, but is only seen with catecholamine secreting tumours.[4] The pathological differential diagnoses for a paraganglioma of the urinary bladder include a granular cell tumour, nested variant of urothelial carcinoma, metastatic large cell neuroendocrine carcinoma and malignant melanoma.[5] The distinctive clinical, histologic and immunohistochemical findings usually permit a definitive diagnosis.

In a series of sixteen cases of paraganglioma of the urinary bladder,[5] of the three patients with malignant tumour, two had metastases at presentation. The time of development of metastases in the third patient is not known. In the present case, distant metastases developed in conjunction with local tumour recurrence in the pelvis.

The treatment of paragangliomas of the urinary bladder is essentially surgical. The preferred approach is tumour excision with partial cystectomy. Total cystectomy is performed in case of infiltrative or multiple tumours.[1], [6] Paragangliomas are generally considered radio- and chemoresistant, but some authors have recommended adjuvant radiotherapy (45 Gy) following surgery, to improve local control.[2]

Naguib et al[7] used 60 Gy to treat bony metastases in a patient with a malignant extraadrenal pheochromocytoma. The present patient was treated with a single dose of palliative radiotherapy to the involved vertebrae. In one series of twelve cases of metastatic pheochromocytoma treated with 131I-metaiodobenzylguanidine (MIBG) therapy, Rose et al[8] reported complete remission in three patients. In the present case, MIBG was not used as the tumour did not show MIBG uptake.

Chemotherapy regimes that have been tried with variable success include a combination of etoposide, 5-FU and cisplatin. Nakane et al [9] reported complete remission in a single patient with metastatic pheochromocytoma using combination chemotherapy with doxorubicin, cyclophosphamide, vincristine and dacarbazine. The present patient was managed with combination chemotherapy using carboplatin, etoposide and zoledronate.

To our knowledge, this is the second case of a paraganglioma of the urinary bladder with skeletal metastases.[10] The rapid progression and occurrence of metastases demonstrates the aggressive course that this apparently benign disease may occasionally take. On account of the rarity of its occurrence, therapeutic guidelines for the management of metastatic disease are not available. The malignant behaviour of the paraganglioma of urinary bladder in the present case shows that meticulous follow up needs to be practiced for all such patients.

   References Top

1.Pastor-Guzman JM, Lopez-Garcia S, Gimenez-Bachs JM, Ruiz-Mondejar R, Canamares-Pabolaza L, Atienzar-Tobarra M et al. Paraganglioma of the bladder: controversy regarding treatment. Urol Int 2004;73:270-5.  Back to cited text no. 1    
2.North CA, Zinreich ES, Christensen WN, North RB. Multiple spinal metastases from paraganglioma. Cancer 1990;66:2224-8.  Back to cited text no. 2  [PUBMED]  
3.Punekar S, Gulanikar A, Sobti MK, Sane SY, Pardanani DS. Pheochromocytoma of the urinary bladder (report of 2 cases with review of literature). J Postgrad Med 1989; 35:909-12.  Back to cited text no. 3    
4.Nesi G, Vezzosi V, Amorosi A, Menghetti I, Selli C. Paraganglioma of the urinary bladder. Urol Int 1996;56:250-3.  Back to cited text no. 4  [PUBMED]  
5.Cheng L, Leibovich BC, Cheville JC, Ramnani DM, Sebo TJ, Neumann RM, et al. Pheochromocytoma of the urinary bladder. Can biologic potential be predicted? Cancer 2000;88:844-52.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Piedrola G, Lopez E, Rueda MD, Lopez R, Serrano J, Sancho M. Malignant pheochromocytoma of the bladder: current controversies. Eur Urol 1997;31:122-5.  Back to cited text no. 6  [PUBMED]  
7.Naguib M, Caceres M, Thomas C. Radiation treatment of recurrent pheochromocytoma of the bladder. Am J Clin Oncol 2002;25:42-4.  Back to cited text no. 7    
8.Rose B, Matthay KK, Price D, Huberty J, Klencke B, Norton JA et al. High-dose 131I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma. Cancer 2003;98:239-48.  Back to cited text no. 8    
9.Nakane M, Takahashi S, Sekine I, Fukui I, Koizumi M, Kage K et al. Successful treatment of malignant pheochromocytoma with combination chemotherapy containing anthracycline. Ann Oncol 2003;14:1449-51.  Back to cited text no. 9    
10.Taue R, Takigawa H, Sinotou K, Uno S, Mori R, Tatara K et al. A case of pelvic malignant paraganglioma. Int J Urol 2001;8:715-8.  Back to cited text no. 10    


  [Figure - 1], [Figure - 2]

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