Triple jeopardy: Blind-ending ureter harboring calculus, ureteral tumor with severe hydronephrosis

INTRODUCTION

The kidney is one of the body’s vital organs for survival, which can be endangered by hydronephrosis (HN), a complication or a consequence arising often from obstructive ureteral pathologies. The blind-ending ureter, ureteral calculus, and ureteral tumor form a triple jeopardy. Each of these issues can individually cause HN, thus putting a kidney at risk of developing end-stage renal disease. The upper tract urothelial carcinoma (UTUC) is a malignancy arising from the epithelium of renal calyces, renal pelvis, and ureter. It is a relatively uncommon disease and accounts for 5-10 % of all urothelial cancers.¹ Ureteric cancers are rare; they are less common than tumors of the renal pelvis and 10 times less common than urinary bladder tumors.² It has an annual incidence of 0.95-1.15/100,000 person/year, more common in men than in women with a male-to-female ratio of 2:1, and are almost always urothelial in origin (>90%), especially the papillary-form.^2,3 The foremost symptoms of ureteral cancer are microscopic or gross hematuria (75-90%), followed by flank pain (8-40%), which is usually caused by HN.^1,4 Other symptoms, such as weight loss, anorexia, fatigue, fever, and night sweats, are uncommon and often associated with advanced/metastatic disease.

Longstanding upper tract stones, on the other hand, remain a major cause of morbidity in the form of pain, infection, and functional impairment of the ipsilateral kidney. Squamous cell carcinoma (SCC) of the upper urinary tract is associated with stone disease and chronic infection, but the association with urothelial carcinoma (UC) has not been proven.⁵ It is hypothesized that chronic irritation of the urothelium may result in squamous metaplasia, which can later develop into SCC. The occurrence of UTUC with calculus is scarcely reported in the literature. Aggarwal et al.⁶(2021) described a large distal ureteric stone and UC of the entire right ureter and renal pelvis, while Kim et al.⁷(2009) reported staghorn stones combined with UC of the renal pelvis. However, the co-existence of these dual pathologies in a blind-ending single ureter tagged as a triple jeopardy to the best of our knowledge had not been reported at the time of our literature search.

This form of presentation poses not just a diagnostic challenge but also a therapeutic dilemma.

Radical nephroureterectomy with bladder cuff excision (BCE) has been the generally acceptable/standard care in the treatment for upper urinary tract tumors because of its superior prognostic value in terms of the low cancer-specific mortality rates, while chemotherapy is often added to the treatment of locally advanced disease.^8,9 The finding of a ureteral tumor in a blind-ending ureter is such a unique situation since the ureteral insertion to the bladder is anatomically absent. This presents a distinctive challenge where the surgeon can only offer nephroureterectomy without the BCE, and this may generate debate in the surgical community, especially among the urologists. The objective of this clinical case is to report our experience in the care of this patient and to add to the body of knowledge on this condition.

CASE REPORT

A 70-year-old retired farmer presented with colicky left flank pain and swelling for 10 months duration, associated with fever, anorexia, weight loss, body weakness, nausea, and vomiting. There was no history of childhood terminal hematuria, cigarette smoking, or exposure to chemicals. The physical examination noted a chronically ill-looking elderly man, febrile (38.5°C), pale, and dehydrated without pedal edema. The respiratory rate, pulse rate, and blood pressure were 28 cycles/min, 100 beats/min, and 140/70 mmHg, respectively. The abdomen was asymmetrically distended, with vague tenderness marked over the left lumbar area.

The laboratory investigation showed a complete blood cell count of 9.0 × 10⁹/L and packed cell blood volume of 32%, which dropped to 28% after rehydration, and serum urea, creatinine, and electrolyte parameters were within normal limits. The urinalysis was unremarkable. The nephrostomy tube urine yielded growth of Candida albicans resistant to biospecies, probably due to prior antibiotics treatment from other facilities and before nephrostomy tube insertion. His prostate-specific antigen was normal (3.2 ng/mL).

The transabdominal ultrasound scan revealed a severe left pyoureteronephrosis and a normal right kidney. The computerized tomography scan (CT-scan) done after left nephrostomy tube insertion revealed a huge left distal impacted ureteral calculus measuring 4 cm x 2.5 cm, a left non-functioning kidney with severe hydroureteronephrosis, and a functioning right kidney. There was no bladder pathology, and the laboratory tests were unremarkable. The clinical and imaging findings of the patient have been shown in Figure 1.

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Figure 1:

Clinical presentation and imaging findings showing (a) Left percutaneous nephrostomy tube insitu (yellow arrow) draining dark effluent (red arrow), (b) Abdominal USS showing left dilated renal calyces (yellow arrow), (c) CT Scan showing left lower ureteral stone (green arrow) and nephrostomy tube insitu (yellow arrow), (d) CT Scan showing normal bladder, catheter balloon, and lower ureteral stone surrounded by ureteral tumour (Rt→Lt) (blue, yellow, and red arrows), (e) Contrast CT Scan of image in figure 1d, (f) Contrast CT Scan showing right normal kidney (blue arrow) and left hydronephrotic kidney (red arrow) with nephrostomy tube insitu (yellow arrow). CT: Computed tomography.

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He was resuscitated, including preoperative blood transfusion of two (2) units and an additional unit intraoperative. He was treated for urosepsis and had an initial insertion of the left percutaneous nephrostomy tube. The nephrostomy tube initially drained up to three (3) liters after insertion, but daily output progressively reduced to 100 mL after 24 hours, and even less than 50 mL. He was operated on by the end of the second week. The 99mTc EC Renal scan/DMSA renal scan was not done to ascertain any function in the left kidney because of the non-availability at the health facility and the high cost of the study. Also, the specimen requires transport to a distant facility. Based on the abdominal CT scan findings of an infected non-functioning left kidney, he was counselled for exploratory laparotomy with left simple nephrectomy. However, the incidental intraoperative findings of a huge ureteral tumor with ureteral calculus within a blind ending single left ureter and hydronephrotic left kidney led to the intraoperative decision to offer the patient left nephroureterectomy, as shown in Figure 2. The histology report confirmed the mass to be invasive papillary ureteral urothelial cell carcinoma, Word Health Organization (WHO)/International Society of Urological Pathology (ISUP) Grade 3 [Figure 3]. He did well postoperatively and was scheduled for adjuvant chemotherapy. Unfortunately, the patient defaulted and was lost to follow-up.

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Figure 2:

Operative findings showing (a) Mobilization of ureteral tumour (yellow arrow), (b) Ureteral tumour in blind-ending ureter (yellow arrow) and left hydronephrotic kidney (blue arrow), (c) Incised tip of blind-ending ureter revealing stone insitu (white arrow), (d) Distal lower ureteral stone (green arrow).

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Figure 3:

Macroscopic and microscopic examination findings showing (a) The gross appearance of the kidney (blue arrow) and attached ureter (white arrow), (b) Cut sections through the kidney shows cystic areas with thin wall (blue arrows) residual renal tissue (white arrow), (c) Cut sections through the kidney shows cystic areas with thin wall (black arrow) and stones (blue arrow), (d) Cut sections through the ureter shows grey white solid tumor (black arrows) obliterating the lumen with a dark stone (white arrow) at the tip (blind end) of the ureter. Blue arrow indicates the proximal part of the ureter, (e) Section of the stone noticed at the blind tip of the ureter, (f) Section shows an invasive tumour growing in papillary pattern (green arrow) with fibrovascular core (yellow arrow) surrounded by dense fibrous tissue (black arrow). H & E, x200, (g) Section shows tumour cells having nuclei which exhibit moderate pleomorphism (yellow arrow), hyperchromatic (yellow arrow) to vesicular nuclei (red arrow) with occasional nucleoli (red arrow) and scanty cytoplasm. H & E, x400. H&E: Hematoxylin and eosin stain.

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DISCUSSION

The upper urinary tract can be afflicted by many diseases, ranging from congenital anomalies and benign and malignant conditions. Ureteral calculus and carcinoma are benign and malignant conditions, respectively, which seldom co-exist. While ureteral calculi are relatively common in our environment, the same cannot be said of ureteral tumors because of their rarity.¹⁰ The blind-ending ureter, which is a result of abnormal embryogenesis, is almost always a component of bifid or duplicated ureter, and approximately 200 cases have been reported in the literature.¹¹ It is a rare congenital anomaly, which is usually asymptomatic but occasionally gives rise to symptoms when complicated by recurrent infections, stone formation, HN, and more rarely malignant transformation.¹²

Halim et al.^13,14 (2005, 2010)and Caliskan et al.¹⁵ (2016) both reported stone formation in the blind ending ureters in the setting of bifid ureters. UC is an uncommon condition even in well-formed single ureters, and following our literature search, there have been no reports of its co-existence with ureteral stone in the setting of a blind-ending single ureter as in our index case. Kawamura et al.¹⁶ (1998) and Kodama et al.¹⁷ (2012) described transitional cell carcinoma in the blind ending branch of bifid ureters but without stone. Our index case was complicated by hydroureteronephrosis and a non-functioning ipsilateral kidney as detected by the imaging studies done, a condition that can occur due to any of the three underlying triple jeopardy, namely the blind-ending single ureter, ureteral tumor, and ureteral calculus. A long-term adverse effect of the blind-ending ureter is stone formation from urinary stasis. In addition, chronic urinary stasis and irritation to the urothelium with subsequent dysplasia may explain the pathogenesis of the malignancy. This pathological process may offer a plausible explanation for what was found in the index case.

Our index patient was offered open nephroureterectomy without BCE, which is assumed to be the standard care in this instance where there is no anatomic connection of the ureter to the bladder. The histology report came out to be invasive papillary UTUC, WHO/ ISUP Grade 3, which are both the most common histological types. Unfortunately, the long term outcomes of the above mentioned treatment could not be assessed as the patient was lost to follow-up due to financial constraints, probably due to lack of insurance coverage for treatment.

CONCLUSION

The coexistence of dual pathologies of ureteral tumor and calculus is an unfamiliar occurrence, more so in a blind ending ureter complicated by HN. This is indeed a triple jeopardy that can lead to end-stage renal replacement therapy if the function of the ipsilateral kidney is not optimal. Its diagnosis can be challenging, and out-of-pocket payment for treatment can be a compounding factor. Therefore, the government and philanthropists in our environment need to do more to change the ugly trend of patients footing the bill for their medical expenses.