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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 3  |  Page : 119-122

Primary ureteral mucinous adenocarcinoma with xanthogranulomatous pyelonephritis masquerading as a giant renal mass


1 Department of Urology, Jupiter Hospital, Thane, Maharashtra, India
2 Department of Pathology, Jupiter Hospital, Thane, Maharashtra, India
3 Department of Nephrology, Jupiter Hospital, Thane, Maharashtra, India

Date of Submission24-Dec-2020
Date of Decision08-Feb-2020
Date of Acceptance21-Jun-2021
Date of Web Publication14-Dec-2021

Correspondence Address:
Ajay Shyam Kanbur
2/101 Wimbledon Park, Pokhran Road 1, Near Cadbury India, Thane - 400 606, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/oji.oji_53_20

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  Abstract 


Primary ureteral carcinoma is rare among all urogenital malignancies, with transitional cell carcinoma being the most common histology followed by squamous cell carcinoma. Adenocarcinoma is extremely rare. Herein, we report a case of primary mucin secreting adenocarcinoma of the right lower ureter in a 67-year-old male. Contrast-enhanced computed tomography (CECT) scan showed a large cystic mass occupying the whole abdomen along with a mass in the lower third of the right ureter mimicking as a giant renal mass. Ureteroscopy followed by laparotomy including right nephroureterectomy with bladder cuff excision was performed. On histopathological examination, the CECT scan-based suspected giant renal mass revealed to be xanthogranulomatous pyelonephritis of the kidney along with primary mucin secreting adenocarcinoma of the ureter.

Keywords: Giant renal mass, mucinous adenocarcinoma of ureter, xanthogranulomatous pyelonephritis


How to cite this article:
Kanbur AS, Mokashi AJ, Dutta S, Hase AN. Primary ureteral mucinous adenocarcinoma with xanthogranulomatous pyelonephritis masquerading as a giant renal mass. Oncol J India 2021;5:119-22

How to cite this URL:
Kanbur AS, Mokashi AJ, Dutta S, Hase AN. Primary ureteral mucinous adenocarcinoma with xanthogranulomatous pyelonephritis masquerading as a giant renal mass. Oncol J India [serial online] 2021 [cited 2022 Jan 21];5:119-22. Available from: https://www.ojionline.org/text.asp?2021/5/3/119/332512




  Introduction Top


Malignancies of the ureter are rare and constitute 0.9%–1.6% of all urogenital cancers. Histologically, transitional cell carcinoma is the most common variant (around 90%) followed by squamous cell carcinoma (<10%). Primary adenocarcinoma is the rarest histological variant that accounts for <1% of all the ureteral malignancies.[1]

Urinary tract adenocarcinomas are divided into three variants such as tubulovillous, mucinous, and papillary nonintestinal. The first two variants represent intestinal adenocarcinomas. Mucinous variants may result from intestinal metaplasia of the transitional epithelium with a better prognosis.[2],[3] The factors such as chronic irritation, inflammation, infection, hydronephrosis, and urinary calculi may lead to glandular metaplasia of the urothelium which can gradually progress to dysplasia and adenocarcinoma.[3]

We report a case of primary mucinous adenocarcinoma of the lower ureter with the co-existence of xanthogranulomatous pyelonephritis (XGP) of the right kidney.


  Case Report Top


A 67-year-old male presented with gradual distension of the abdomen, with dyspnea on exertion Grade 1. He was not a diabetic and was on hypertension medication. Clinically, his vitals were normal. He had pallor and grossly distended tense abdominal mass occupying the entire abdomen. It was nontender. There was no history of urological disease. Investigations revealed hemoglobin of 7 g% and serum creatinine level of 1.1 mg%. Urine routine and microscopy were within the normal limit, and culture sensitivity revealed no bacterial growth. Urine cytology revealed the absence of any malignant cells. Ultrasonography of the abdomen showed a large cystic mass replacing the right kidney and the left kidney was normal. Contrast-enhanced computed tomography (CECT) scan showed a large cystic mass occupying the right-side whole abdomen, with a staghorn stone in the right renal pelvis and a mass in the lower third of the right ureter about 4 cm away from the right ureterovesical junction [Figure 1]. There were no enlarged lymph nodes in the abdomen on imaging. DTPA scan showed no uptake from the right kidney and the left kidney was normal. Positron-emission tomography–computed tomography (PET CT) showed metabolic uptake lesion only in the right lower ureter.
Figure 1: A large cystic lesion occupying the right side whole abdomen with a staghorn stone in the right renal pelvis and mass in right ureter shown as asteric

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The patient was subjected to ureteroscopy and laparotomy with right nephroureterectomy with bladder cuff excision. At ureteroscopy, the right orifice was obscured by a gelatinous substance, making vision difficult and hence could not be accessed. At operation, the entire abdomen and pelvis was occupied by a cystic mass, pushing the right colon across the midline, putting the mesocolon under stretch. It was adherent to the liver, diaphragm, and major vessels. For easier dissection, the fluid contents of the mass were aspirated. This had dark hemorrhagic fluid full of mucous and thick gelatin. About 10 L of fluid material was aspirated [Figure 2]. The frozen section of the cystic mass revealed XGP. Right kidney, entire ureter including 4 cm of tumor bearing portion with bladder cuff was excised as a single unit. There were no enlarged lymph nodes in the retroperitoneum. Gross specimen revealed the enlarged right kidney replaced by thinned-out cortex and medulla with cystic spaces and necrotic tissues and a mass in the lower third of the ureter [Figure 3]a, [Figure 3]b, [Figure 3]c. There was a presence of staghorn calculus in the right renal pelvis [Figure 3]d.
Figure 2: Gelatinous substance from the right kidney

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Figure 3: Gross specimen showing (a) enlarged kidney replaced by thinned-out cortex–medulla with cystic spaces and necrotic tissues, and mass in the lower part of the ureter, (b and c) right lower ureteric mass, and (d) staghorn stone in the right renal pelvis

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Histopathological examination of the resected cystic mass revealed XGP Stage II with foamy cells and cholesterol clefts. The ureteral mass on histopathological examination revealed adenocarcinoma in tubular and small papillary pattern and lined by intestinal-type mucin containing cells with mucin on the surface [Figure 4]. The mucous membrane was positive for beta-catenin on immunohistochemistry. With the above findings, the case was diagnosed as a well-differentiated mucinous adenocarcinoma of the right lower ureter with XGP of the right kidney postoperative. The patient made a good recovery after having lost about 18 kg. He received six cycles of adjuvant chemotherapy with gemcitabine and cisplatin-based regimen and was doing well without any evidence of disease till 6 months after completion of the same. Thereafter. he shifted to his hometown and was lost to follow-up.
Figure 4: Histopathological examination on H and E stain (×10) showing well-differentiated mucinous adenocarcinoma with tubular and papillary pattern, the presence of intestinal type of cells, and pools of mucin seen in the lamina with uninvolved muscularis

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  Discussion Top


Primary adenocarcinoma of the upper urinary tract, especially the ureter, is rarely reported. Most of the cases occur in the seventh decade of life, with 45% being in the lower third of the ureter and 40% associated with calculus.[1] Due to the paucity of data, the pathogenesis, diagnosis, and optimal treatment are not clear. The process of development of adenocarcinoma of the ureter is not clear. However, metaplasia of the epithelium of the urinary tract is a commonly accepted mechanism. Few reports suggested the occurrence of mucinous adenocarcinoma of the upper urinary tract following glandular metaplasia of the transitional epithelium due to long-standing chronic inflammation of the urinary tract or urinary calculi.[3] Our case of mucinous adenocarcinoma of ureter, presence of renal stones suggest a possible intestinal metaplasia of the transitional epithelium resulting mucinous adenocarcinoma.

Hematuria, low back pain, abdominal pain, and urinary disturbance are common presentation. Preoperative diagnosis is difficult and most of these patients are diagnosed on pathological examination after surgery. This is because symptoms as the result of ureteral tumors were masked by the effect of commonly associated urinary calculi or pyonephrosis, and in case of clearly defined tumor, surgery was performed without biopsy.[4]

Surgery is the treatment of choice for upper urinary tract cancers and the radical nephroureterectomy with the removal of bladder cuff is the common surgical procedure as performed in our case. The bladder cuff should be removed because of high rates of ureteral stump recurrence in up to 30%–75% of the patients undergoing radical surgery.[5] Adjuvant radiation should be given in cases with close or positive margins to prevent locoregional recurrence. Previously, chemotherapeutic regimen of paclitaxel plus carboplatin combination and methotrexate, vinblastin, adriamycin, and cisplatin regimen have been used.[1] Lack of effective adjuvant radiotherapy and no established effective chemotherapeutic regimens has resulted in poor outcome necessitating careful follow-up after definitive surgery.[4] Moreover, survival in upper urinary tract adenocarcinoma depends on histopathological subtypes with a 5-year survival rate of <30% in tubulovillous variants, 67% survival rate in mucinous tumors, and a nearly 100% survival rate in papillary nonintestinal tumors.[6] In our case, the patient received adjuvant chemotherapy with six cycles of gemcitabine and cisplatin-based combination regimen and is without any evidence of disease at his 6-month follow-up after completion of treatment.

Moreover, the presence of XGP makes a diagnostic dilemma with malignancy of kidney and is difficult to differentiate preoperatively as seen in our case.[7] XGP patients are commonly presented with fever, abdominal and/or flank pain, weight loss, malaise, anorexia, and lower urinary tract symptoms. Leukocytosis, anemia, and increased elevated sedimentation rate are seen in the majority of XGP patients. Pyuria is commonly seen in XGP patients (60%–90%) and urine cultures are usually positive at the time of diagnoses.[8] However, in our case, pyuria was absent and urine culture was negative for pathogens. Our case on ultrasonography and CECT scan revealed a large cystic mass replacing the right kidney along with right ureteral mass suspecting as a case of giant renal mass. However, PETCT scan evaluation in our case gives only clue of ureteral mass without any renal mass. Postoperative pathological examination of our case came out to be XGP of the kidney with mucinous adenocarcinoma of the same site ureter. Such concurrent finding of XGP of the same site kidney along with mucinous adenocarcinoma of ureter without the involvement of renal pelvis is unique one and was not reported previously.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


  Conclusion Top


Primary mucinous adenocarcinoma of ureter is rare. This coupled with giant kidney with mucin and gelatin secreting intestinal metaplasia with underlying XGP is rare and anecdotal. PETCT scan evaluation could help to differentiate the presence of XGP from kidney mass, but radical nephroureterectomy is the ultimate goal both for diagnostic and therapeutic purpose.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chaudhary P, Agarwal R, Srinivasan S, Singh D. Primary adenocarcinoma of ureter: A rare histopathological variant. Urol Ann 2016;8:357-9.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Abt D, Müllhaupt G, Mordasini L, Diener PA, Schmid HP. Metachronous primary adenocarcinoma of distal and proximal ureter within two years. Case Rep Urol 2014;2014:659258.  Back to cited text no. 2
    
3.
Raphael V, Sailo S, Bhuyan A, Phukan M. Mucinous adenocarcinoma of the renal pelvis with adenocarcinoma in situ of the ureter. Urol Ann 2011;3:164-6.  Back to cited text no. 3
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4.
Zennami K, Yamada Y, Nakamura K, Katuda R, Tobiume M, Naruse K, et al. Primary adenocarcinoma of the ureter: Report of a case. Hinyokika Kiyo 2007;53:645-8.  Back to cited text no. 4
    
5.
Rubenstein MA, Walz BJ, Bucy JG. Transitional cell carcinoma of the kidney 25-year experience. J Urol 1978;119:594-7.  Back to cited text no. 5
    
6.
Spires SE, Banks ER, Cibull ML, Munch L, Delworth M, Alexander NJ. Adenocarcinoma of renal pelvis. Arch Pathol Lab Med 1993;117:1156-60.  Back to cited text no. 6
    
7.
Zorzos I, Moutzouris V, Petraki C, Katsou G. Xanthogranulomatous pyelonephritis–The “great imitator” justifies its name. Scand J Urol Nephrol 2002;36:74-6.  Back to cited text no. 7
    
8.
Dell'Aprovitola N, Guarino S, Del Vecchio W, Camera L, Chiancone F, Imbimbo C, et al. Xanthogranulomatous pyelonephritis mimicking a renal cell carcinoma: A unique and challenging case. Acta Radiol Short Rep 2014;3:2047981613513763.  Back to cited text no. 8
    


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



 

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