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Year : 2011  |  Volume : 54  |  Issue : 2  |  Page : 405-406
'Functional' paraganglioma of ureter: An unusual case

1 Department of Pathology, Chhatrapati Shahuji Maharaj Medical University (CSMMU), Lucknow, India
2 Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
3 Department of Urology, (CSMMU), Lucknow, India

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Date of Web Publication27-May-2011

How to cite this article:
Awasthi NP, Kumari N, Krishnani N, Goel A. 'Functional' paraganglioma of ureter: An unusual case. Indian J Pathol Microbiol 2011;54:405-6

How to cite this URL:
Awasthi NP, Kumari N, Krishnani N, Goel A. 'Functional' paraganglioma of ureter: An unusual case. Indian J Pathol Microbiol [serial online] 2011 [cited 2021 Nov 28];54:405-6. Available from: https://www.ijpmonline.org/text.asp?2011/54/2/405/81631

The most common tumor of urinary tract is urothelial carcinoma. Paragangliomas of urinary tract are very rare and form less than 0.06% of all urinary bladder tumors and less than 6% of all paragangliomas. [1] Paraganglioma of ureter is very rare with only one case reported in the English literature. [2] The present case is a functional paraganglioma of ureter and showed no recurrence after two years of follow up after surgical management.

A 33-year-old male presented with recurrent attacks of palpitation and episodic hypertension for two years. Ultrasonography showed a left-sided infra-renal mass with dilated renal pelvis. Computed tomography (CT) scan showed the mass to be located in the ureter [Figure 1]. In view of typical clinical presentation, the patient was investigated for evidence of functional paraganglioma of the ureter. Urinary vanillylmandelic acid (VMA) was slightly increased (6.9 mg/24 h; range 1.4-6.5), while 24-h urinary metanephrine was normal. In view of these findings, a pre-operative diagnosis of functional paraganglioma of ureter was kept. On surgical exploration, the tumor was 6 × 5 × 4 cm in size and the involved ureter along with 1-cm healthy margins was excised. Microscopically, the tumor cells were arranged in vague nests, sheets, fascicles and showed spindling at places, had mildly anisomorphic vesicular nuclei and granular cytoplasm [Figure 2]a and b. The tumor was seen within the wall of the ureter [Figure 3]. No mitosis or necrosis was seen. Immunohistochemistry (IHC) for chromogranin, neuron-specific enolase (NSE), smooth muscle actin (SMA), desmin, cytokeratin, vimentin, S-100 and HMB45 showed positivity for NSE and chromogranin. [Figure 4] IHC was negative for all other markers. There is no recurrence after two years of follow-up.
Figure 1: Contrast-enhanced CT scan image showing tumor in the left ureter

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Figure 2: a: Tumor cells are arranged in sheets and fascicles comprising of polygonal and spindle cells (H and E, ×200)
Figure 2: b: Tumor cells showing showing vesicular nucleus and granular cytoplasm (H and E, ×400)

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Figure 3: Tumor is seen within the wall of ureter showing transitional epithelium towards lumen and tumor in the smooth muscle layer (H and E, ×40). Inset shows high power view of the transitional epithelium (H and E, ×400)

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Figure 4: Immunohistochemistry for chromogranin showing positivity in tumor cells (IHC, ×400)

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Paraganglioma is the generic term for tumors arising from extra-adrenal chromaffin cells situated within sympathetic and parasympathetic system. Paraganglioma of the urinary bladder was first reported by Zimmerman [3] in 1953. The classic triad of episodic hypertension, persistent hematuria and post-micturition syncope is virtually diagnostic of paraganglioma; however, it is seen only in catecholamine-secreting tumors. [1] Tumors arising from sympathetic ganglia are hormonally active and arise in retroperitoneum. Sixty percent of the retroperitoneal paragangliomas are functional. When a nested pattern is seen in urinary tract tumors, the differential varies from nested variant of transitional cell carcinoma, metastatic renal cell carcinoma, paraganglioma, malignant melanoma, granular cell tumor, carcinoid and large cell neuroendocrine carcinoma. [4] Sometimes in paragangliomas, the diffuse pattern of growth, nuclear atypia and pleomorphism, tumor necrosis and dysplasia in the overlying epithelium may further lead to mis-diagnosis of conventional urothelial carcinoma. However, paragangliomas are usually lobulated and well circumscribed. [5] Careful assessment of routine histology may raise the suspicion of paraganglioma and prompt immunohistochemical evaluation. Paragangliomas are positive for neuroendocrine markers like chromogranin, whereas urothelial carcinomas are negative. Renal cell carcinomas may sometimes have nested pattern and granular cytoplasm and are positive for epithelial markers and renal cell carcinoma antigen but are negative for neuro-endocrine markers. Melanomas are great mimickers of variety of tumors and the melanin pigment does not necessarily prove diagnosis of melanoma as pigmented paragangliomas do occur. [4] Melanomas show positivity for markers like HMB-45, Melan A and MiTF, whereas paragangliomas are negative for these. Carcinoid and other neuroendocrine tumors are very rare in urinary tract and apart from neuro-endocrine markers, they are positive for cytokeratin in contrast to paragangliomas. Granular cell tumors are positive for S-100 in contrast to paraganglioma where sustentacular cells show positivity for S-100. Malignancy is uncommon and there is no definite histological criterion to differentiate between benign and malignant counterparts. The only definitive sign of malignancy is metastasis. Careful clinical evaluation including history of recurrent attacks of palpitation, episodic hypertension, syncopal attack and biochemical evaluation including VMA and histological examination combined with a panel of immunohistochemical markers may help to differentiate paraganglioma from other tumors.

   References Top

1.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. 1
2.Kumar PB, Natarajan K, Hegde P. Paraganglioma of ureter. The Internet J Urol 2010;7:1.  Back to cited text no. 2
3.Zimmerman IJ, Biron RE, Macmahon HE. Pheochromocytoma of the urinary bladder N Engl J Med 1953;249:25-6.   Back to cited text no. 3
4.Zhou M, Epstein JI, Young RH. Paraganglioma of the urinary bladder: A lesion that may be misdiagnosed as urothelial carcinoma in transurethral resection specimens Am J Surg Pathol 2004;28:94-100.  Back to cited text no. 4
5.Grignon DJ. Neoplasms of the urinary bladder. In: Bostwick DG, Eble, JN, editors. Urologic Surgical Pathology. St. Louis: Mosby-Year Book; 1997. p. 214-305.  Back to cited text no. 5

Correspondence Address:
Namrata P Awasthi
Hig 48, Sector E, Aliganj, Lucknow, 226 024, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.81631

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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