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Year : 2017  |  Volume : 6  |  Issue : 2  |  Page : 118-120

Paraganglioma of ureter: A case report with review of literature

1 Department of Pathology, Rangaraya Medical College, Amalapuram, East Godavari District, Andhra Pradesh, India
2 Konaseema Institute of Medical Sciences, Amalapuram, East Godavari District, Andhra Pradesh, India

Date of Web Publication13-Jun-2017

Correspondence Address:
Lakshmi V Chavali
Department of Pathology, Rangaraya Medical College, Opp. GGH, Kakinada - 533 004, East Godavari District, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2277-8632.208001

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Keywords: Paragangliomas, Extradrenal tumors, Ureteric paraganglioma

How to cite this article:
Chavali LV, Rao BR. Paraganglioma of ureter: A case report with review of literature. J NTR Univ Health Sci 2017;6:118-20

How to cite this URL:
Chavali LV, Rao BR. Paraganglioma of ureter: A case report with review of literature. J NTR Univ Health Sci [serial online] 2017 [cited 2021 May 11];6:118-20. Available from: https://www.jdrntruhs.org/text.asp?2017/6/2/118/208001

  Introduction Top

Paragangliomas in the genitourinary tract have been described in the kidney, bladder, prostate, urethra, and spermatic cord. Lesions occurring in the ureter are rare and review of available literature revealed four cases to have been reported so far with the first case being reported in 1970. Most of these cases were symptomatic and functional. Herein, we report a case of non-functional paraganglioma of the ureter which was identified only after histopathologic examination of the tumor.

  Case Report Top

A 40-year-old woman presented with attacks of pain abdomen. Examination revealed a normotensive nondiabetic patient with flank pain. Ultrasonography showed hydronephrosis of the left kidney and a small hypoechoic mass arising from the left ureter. All other routine hematological investigations were within normal limits.

Intravenous pyelography (IVP) revealed mild hydroureteronephrosis with hold up of contrast extending up to L3 level. There was no obvious radiodensity demonstrable suggestive of a stricture or radiolucent calculus. Biochemical investigations including blood sugar, urea, and serum creatinine were within normal limits. Specific investigations suggestive of pheochromocytoma, such as serum epinephrine and norepinephrine or urinary vanillyl mandelic acid, were not done as the lesion was not suspected.

Further imaging studies were not done, but the patient was subjected to surgery for investigating the cause of hydronephrosis.

Intraoperatively, the surgeon found a small mass arising from the mid segment of the left ureter, with an enlarged hydronephrotic left kidney. The mass was resected along with a segment of the ureter. The postoperative period was uneventful and the patient was lost to follow-up after 1 year.

We received a 3-cm-long segment of ureter with a 1.5-cm-diameter, globular, gray brown soft tissue mass arising from it. Cut section revealed a well-encapsulated gray brown mass with foci of hemorrhages.

Histopathology revealed a well-circumscribed vascular tumor in the adventitia and muscle coat of the ureter [Figure 1]. The tumor was composed of large polygonal cells with abundant eosinophilic and faintly granular cytoplasm with cytoplasmic vacuoles in some. The nucleus was vesicular with finely clumped chromatin and occasional nucleoli. Focal areas showed hyperchromatic and pleomorphic nuclei. Mitotic activity was scarce. The cells were arranged in anastomosing sheets, nests, and organoid pattern [Figure 2]. No foci of lymphovascular invasion were seen.
Figure 1: Scanner view showing ureter with tumor tissue in its wall

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Figure 2: Low-power view of H&E stained section showing polygonal cells with eosinophilic and granular cytoplasm in nests

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A differential diagnosis of paraganglioma/glomus tumor/hemangiopericytoma/carcinoid was made and immunohistochemistry (IHC) was done. IHC revealed diffuse cytoplasmic positivity with chromogranin [Figure 3] and positivity in the cells at the periphery of the nests with S-100. A diagnosis of paraganglioma of the ureter was made.
Figure 3: High-power view of immunohistochemical stain showing diffuse chromogranin positivity

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  Discussion Top

Paragangliomas are tumors arising from the paraganglionic tissue. They are classified on the basis of the anatomic region of origin. Those arising from the adrenal medulla are known as pheochromocytomas, while the rest are labeled paragangliomas.

These extra-adrenal paragangliomas are further classified into tumors arising in the head and neck region including those from the aorticopulmonary paraganglia and tumors from the sympathoadrenal neuroendocrine system.

The head and neck region tumors are non-functional tumors of the parasympathetic system, often noticed because of their mass effect.

Tumors below the neck often arise from the sympathetic system and are frequently functional. In the retroperitoneum, extra-adrenal tumors often arise from the paraortic sympathetic chain, with around 60% of them being functional. Twenty percent of them are discovered incidentally. In the genitourinary tract, bladder is the most common site of occurrence, with over 250 cases having been reported so far. Tumors have also been described to arise in the kidney, urethra, prostate, and spermatic cord.[1]

Paragangliomas arising from the ureter are rare, with only four cases having been described so far.[2],[3],[4],[5] Four more cases of tumors arising around the ureter and involving the bladder or adjacent tissues have been reported.[3],[6],[7] The age range extended from the second to seventh decade of life and involved both the sexes. Three of these four ureteric neoplasms described were functional and presented with symptoms related to the products liberated by the tumor.[2],[3],[4] The fourth case presented with hematuria, a symptom related to location.[4]

Grossly, they ranged from 3.5 to 6 cm in diameter and occurred at all levels of the ureter, from the hilar region to the site of insertion in the bladder.[2],[3] The gross morphology and histopathology of all the cases described were similar to paragangliomas occurring elsewhere, with all the tumors showing the Zellballen pattern.

The postoperative period in all the four cases was uneventful and follow-up of some these cases over a 2-year period revealed no evidence of recurrence.

Chromaffin tissue was identified in the muscularis and adventitial layers of the ureter by Boyarsky in 1969 and the first case of paraganglioma of ureter was described by Allen and associates in 1970 wherein the lesion was located in the upper third of ureter.[2]

Cabanas and associates reported the second case wherein the tumor presented as a ureterocele. Albores-Savedra and others described two cases of paragangliomas in the bladder occurring near the ureteric orifice, which probably arose from the chromaffin cells in the ureteric wall.[3]

Bladder is the most common site for paragangliomas arising in the genitourinary tract, and malignancy has been described in 15%-20% of these tumors. The course of ureteric paragangliomas identified so far suggests that they are probably benign lesions which can be managed by wide excision. Any generalization regarding their behavior cannot be made in view of the very few cases reported.

  References Top

Urologic Pathology. In: Peterson RO, Sesterhann IA, Davis CJ, editors. Urologic Pathology. 3rd edn. Philadelphia: Lippincott Williams and Williams; 2009. p. 155.  Back to cited text no. 1
Allen SD, Karafin L, Kendall AR. Non-visualization of the kidney due to a ureteral pheochromocytoma. J Urol 1971;105:571-4.  Back to cited text no. 2
Cabañas VY, Faulconer RJ, Fekete AM. Pheochromocytoma presenting as a ureterocele. J Urol 1973;110:389-90.  Back to cited text no. 3
Kumar P, Natarajan K, Hegde P. Paraganglioma of Ureter. Internet J Urol 2009;7:1. Available from: http://www.ispub.com/IJU/7/1/13065 [Last accessed on 2015 Nov 05].  Back to cited text no. 4
Awasthi NP, Kumari N, Krishnani N, Goel A. 'Functional' paraganglioma of ureter: An unusual case. Indian J Pathol Microbiol 2011;54:405-6.  Back to cited text no. 5
[PUBMED]  [Full text]  
Lubina-Solmon A, Papa M, Ilan S. Paraganglioma of the Urinary tract. Society for Endocrinology, National Clinical Cases. www.bioscilibrary.com/.../clinicalcases/ 2008.  Back to cited text no. 6
Saurborn DP, Kruskal JB, Stillman IE, Parangi S. Paraganglioma of ureter- Best cases from the AFIP: Paraganglioma of the Organs of Zuckerkandl. Radiographics 2003;23:1279-86.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]


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