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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 2  |  Issue : 2  |  Page : 115-117

Incidental upper tract urothelial carcinoma presenting as pyonephrosis


Departments of Pathology and Urology, Sri Devaraj Urs Medical College, Kolar, Karnataka, India

Date of Submission13-Mar-2017
Date of Acceptance12-Jun-2017
Date of Web Publication15-Dec-2017

Correspondence Address:
Dr. K Manjula
Sri Devaraj Urs Medical College, Tamaka, Kolar - 563 101, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/bjhs.bjhs_2_17

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  Abstract 


Upper tract urothelial carcinomas (UTUCs) are uncommon and account for only 5%–10% of urothelial carcinomas. The most common symptom of UTUC is gross or microscopic hematuria (70%–80%). A 55-year-old woman admitted with the history of fever and pain in abdomen for the past 20 days. Her history was not significant. She underwent right nephroureterectomy with the provisional clinical diagnosis of pyonephrosis with nonfunctioning kidney. Histopathological examination of nephrectomy specimen showed features of high-grade urothelial carcinoma with renal parenchyma invasion (T3N0M0). Here, we present a rare case of incidental UTUC presenting as pyonephrosis.

Keywords: Hematuria, pyonephrosis, urothelial carcinoma


How to cite this article:
Manjula K, Nagaraj G, Shetty K, Prasad C. Incidental upper tract urothelial carcinoma presenting as pyonephrosis. BLDE Univ J Health Sci 2017;2:115-7

How to cite this URL:
Manjula K, Nagaraj G, Shetty K, Prasad C. Incidental upper tract urothelial carcinoma presenting as pyonephrosis. BLDE Univ J Health Sci [serial online] 2017 [cited 2020 Aug 13];2:115-7. Available from: http://www.bldeujournalhs.in/text.asp?2017/2/2/115/220940



Primary urothelial carcinoma originating in the ureter or renal pelvis is collectively called upper tract urothelial carcinomas (UTUCs). UTUCs are uncommon and account for only 5%–10% of urothelial carcinomas.[1],[2] UTUCs have a peak incidence in older age, and they are three times more prevalent in men than in women.[3] Tobacco and occupational exposure are the main risk factors.[2],[3] The most common symptom of UTUC is gross or microscopic hematuria (70%–80%); other common symptoms are flank pain and presenting as a lumbar mass.[3] Grade and stage are the most important prognostic factors in UTUCs. UTUCs presenting as pyonephrosis in female with kidney invasion are very rare.


  Case Report Top


A 55-year-old woman admitted with the history of fever and pain in abdomen for the past 20 days. Her history was not significant. On examination, she had right hypochondriac and flank tenderness. Urine analysis showed albumin - 1+, pus cells - 10–12/hpf, red blood cell - nil, epithelial cells - 16–18/hpf, and casts/crystals - nil; blood analysis showed blood urea - 40 mg/dl and serum creatinine - 1.3 mg/dl. Ultrasound examination showed gross hydropyonephrosis. The patient underwent right nephroureterectomy with the provisional clinical diagnosis of pyonephrosis with nonfunctioning kidney.

Macroscopy

Right nephrectomy specimen, measured 10 cm × 6 cm × 2 cm, weighed about 96 g. Capsule was thick and adherent. External surface was uneven and showed scars with nodular areas. Cut section showed obliteration of corticomedullary junction and dilatation of pelvicalyceal system [Figure 1]. Also, we noted a well-defined gray-white area measuring about 1.2 cm at the corticomedullary junction. Ureter measured 3 cm. Lumen was dilated, showed papillary excrescence.
Figure 1: Well-defined gray-white area within the nephrectomy specimen

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Microscopy

Sections studied from the well-defined gray-white area revealed tumor cells arranged in papillary architecture; tumor cells were pleomorphic and polygonal in shape with vesicular nucleus, moderate amount of eosinophilic cytoplasm [Figure 2] and [Figure 3]. Occasional mitotic figures were also noted. Sections studied from the ureter also revealed similar features [Figure 4]. Sections studied from the other areas of the kidney showed xanthogranulomatous pyelonephritis. Thus, features were suggestive of high-grade urothelial carcinoma with renal parenchyma invasion (T3N0M0).
Figure 2: Well-defined tumor with papillary architecture within kidney parenchyma (H and E, ×100)

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Figure 3: Polygonal cells with pleomorphic vesicular nucleus, moderate clear to acidophilic cytoplasm (H and E, ×400)

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Figure 4: Ureter with upper tract urothelial carcinoma (H and E, ×100)

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  Discussion Top


Epidemiology of urothelial carcinoma of upper tract is similar to bladder carcinoma but has a different natural history. Sixty percent of UTUCs are invasive at diagnosis, whereas bladder carcinoma invasion at diagnosis accounts to 15%–20%.[2] Long-term high-dose analgesic abuse (phenacetin) produces a nephropathy called capillosclerosis, and it is associated with increased risk of development of UTUCs.[4]  Balkan nephropathy More Details and Chinese herb nephropathy are also associated with the development of UTUCs. Certain plant derivatives endemic to the Balkans have mutagenic action.[2],[5],[6] Certain aromatic amines used in dye industry also act as risk factors. UTUC is also linked to hereditary cancer syndromes such as Lynch syndrome and Muir–Torre syndrome and also to black foot disease.[1],[3]

The most common symptom of UTUC is gross or microscopic hematuria present in more than 75% of patients.[1],[2],[3],[4] Patients may also come with symptoms of flank pain, or flank mass, increased urinary frequency. In 10%–15% of patients, the lesion may be asymptomatic.[2],[3],[5]

UTUC is discovered incidentally in <5% of patients.[6],[7] Unusual clinical manifestations such as our case presenting as incidental tumor with hydronephrosis accounts to 7%. The standard lines of investigation for hematuria are urine for cytology, upper tract imaging, and cystoscopy. Computed tomography urography is the imaging technique with the highest accuracy for UTUCs.[1],[2] Development of hydropyonephrosis indicate bad prognosis. Degree of hydronephrosis independently correlates with a bad prognosis.[8] Tissue diagnosis using ureteroscope before surgery helps in diagnosis as well as patients' future management.[9]

The classification, morphology, and grading scheme of UTUCs are similar to those of bladder carcinomas.[1],[2],[3] Low-grade and low-stage tumors have a more favorable disease course than higher stage and grade. The most important prognostic factors are tumor grade, stage, and patient age.[1],[2],[3],[4],[9] Other important prognostic factors include architecture, lymphovascular invasion, multifocality, and the presence of tumor necrosis. The presence of hydronephrosis, presence of systemic symptoms at presentation, history of urothelial carcinoma of bladder, obesity, and advanced age negatively affect the prognosis in UTUCs.[1],[3],[10]

UTUC is an important differential diagnosis for poorly differentiated carcinomas presenting as a renal mass.[3] Focal presence of urothelial differentiation helps in diagnosis. Immunohistochemistry is of limited value, though co-expression of cytokeratin (CK) 7 and CK20 and/or p63, combines with absence of reactivity for paired box (PAX)-2 or PAX-8, would support urothelial carcinoma.[3]

The gold standard surgical management for localized UTUC is radical nephroureterectomy with bladder cuff excision.[1],[2],[4] Lymphadenectomy is often performed in patients with high-grade invasive tumors.[1] Despite surgical treatment, 5-year cancer-specific mortality rates remain relatively high in these patients. Patients with UTUCs require more intense surveillance, as they have a lifelong increased risk of developing urothelial tumors in the contralateral upper urinary tract or bladder.[11],[12]


  Conclusion Top


UTUCs are rare tumors, can present as pyohydronephrosis, and require intense surveillance because of increased risk of development of metachronous bladder tumors, local recurrence, and distant metastasis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hutchinson R, Haddad A, Sagalowsky A, Margulis V. Upper tract urothelial carcinoma: Special considerations. Clin Adv Hematol Oncol 2016;14:101-9.  Back to cited text no. 1
    
2.
Rouprêt M, Babjuk M, Compérat R, Cowa NC, Kaasinen Zigeuner R, et al. Guidelines on urothelial carcinomas of the upper urinary tract. Eur Urol 2013;63:1059-71.  Back to cited text no. 2
    
3.
Eble JN, Grignon DJ, Young RH. Tumors of the renal pelvis and ureter. Diagnostic Histopathology of Tumors. Philadelphia: Elsevier and Saunders; 2013. p. 603-9.  Back to cited text no. 3
    
4.
Kirkali Z, Tuzel E. Transitional cell carcinoma of the ureter and renal pelvis. Crit Rev Oncol Hematol 2003;47:155-69.  Back to cited text no. 4
    
5.
Colin P, Koenig P, Ouzzane A, Berthon N, Villers A, Biserte J, et al. Environmental factors involved in carcinogenesis of urothelial cell carcinomas of the upper urinary tract. BJU Int 2009;104:1436-40.  Back to cited text no. 5
    
6.
Jain KA. Transitional cell carcinoma of the renal pelvis presenting as pyonephrosis. J Ultrasound Med 2007;26:971-5.  Back to cited text no. 6
    
7.
Prando A, Prando P, Prando D. Urothelial cancer of the renal pelvicaliceal system: Unusual imaging manifestations. Radiographics 2010;30:1553-66.  Back to cited text no. 7
    
8.
Chung PH, Krabbe LM, Darwish OM, Westerman ME, Bagrodia A, Gayed BA, et al. Degree of hydronephrosis predicts adverse pathological features and worse oncologic outcomes in patients with high-grade urothelial carcinoma of the upper urinary tract. Urol Oncol 2014;32:981-8.  Back to cited text no. 8
    
9.
Williams SK, Denton KJ, Minervini A, Oxley J, Khastigir J, Timoney AG, et al. Correlation of upper-tract cytology, retrograde pyelography, ureteroscopic appearance, and ureteroscopic biopsy with histologic examination of upper-tract transitional cell carcinoma. J Endourol 2008;22:71-6.  Back to cited text no. 9
    
10.
Lughezzani G, Burger M, Margulis V, Matin SF, Novara G, Roupret M, et al. Prognostic factors in upper urinary tract urothelial carcinomas: A comprehensive review of the current literature. Eur Urol 2012;62:100-14.  Back to cited text no. 10
    
11.
Chien TM, Li CC, Li WM, Yeh HC, Ke HL, Lee HY, et al. The significant prognosticators of upper tract urothelial carcinoma. Urol Sci 2015;26:230-4.  Back to cited text no. 11
    
12.
Raza SA, Sohaib SA, Sahdev A, Bharwani N, Heenan S, Verma H, et al. Centrally infiltrating renal masses on CT: Differentiatiating intrarenal transitional cell carcinoma from centrally located renal cell carcinoma. Am J Roentgenol 2012;198:846-53.  Back to cited text no. 12
    


    Figures

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



 

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