Ureteral carcinoma is a type of malignancy involving the ureter. The condition is rare, and the vast majority of tumors are transitional cell carcinomas (TCC) of the ureter, classified under the wider category of urothelial tumors of the ureter.
Presentation
Ureteral carcinoma is a tumor that may be located in any part of the ureter. This malignancy is commonly referred to as urothelial tumor of the renal pelvis and ureter; the greatest majority of the neoplasms are of urothelial nature and isolated ureteral cancer is a rare medical occurrence with few pertaining literary documentations [1].
The predominant manifesting symptom of a primary ureteral carcinoma is gross hematuria, often with no other accompanying symptoms, either specific to a urinary tract pathology or constitutional [2]. The second most frequently reported complaint is a flank pain, observed in 1 in 5 patients diagnosed with a ureteral carcinoma [3]. Flank pain can be initially misdiagnosed as originating from an episode of renal colic due to ureterolithiasis, and in a case of ureteral carcinoma, the painful feeling is attributed to blood clots forming in the area. An equal percentage of patients also present with a palpable lumbar mass and less than 10% report dysuric symptoms, such as pain or burning upon urination and an increased frequency to urinate. Generalized manifestations such as weight loss and skeletal pain are rarely documented and are a sign of progressed malignant disease.
A ureteral carcinoma can also occur as a metastatic tumor, in which case most patients report no symptomatology and are diagnosed incidentally [4] [5]. The minority of patients who experience cancer-related symptomatology present with dysuric symptoms consistent with a renal colic due to obstruction (flank pain) or a urinary tract infection. Hematuria is also a symptom of a metastatic ureteral carcinoma and, in the uncommon case where the ureters are bilaterally affected, patients may present with anuria [6] [7].
Entire Body System
- Weakness
[…] type of bladder cancer include: blood in the urine an urgent need to urinate needing to urinate more often than usual pain when you urinate When the cancer spreads, symptoms can include: loss of appetite weight loss lower back pain night sweats fatigue weakness [healthline.com]
He also complained of anorexia, nausea and generalized weakness for 1 week. There was no history of weight loss. There were no voiding or storage symptoms. There was no hematuria. His past medical history was notable for mild bronchial asthma. [omicsonline.org]
Note: In a large series, PSA positivity is reported in 1.4% bladder UCC. [11] In half the cases the staining is weak and in the other half it is strong. [11] IHC for staging Smoothelin immunostain for muscularis propria invasion versus muscularis mucosae [librepathology.org]
Weak or interrupted ("stop-and-go") flow of urine. Frequent urination, especially at night. Incontinence. Discharge from the urethra. Bleeding from the urethra or blood in the urine. A lump or thickness in the perineum or penis. [medicinenet.com]
Digital rectal examination was refused by the patient due to his physical weakness. [tcr.amegroups.com]
- Recurrent Urinary Tract Infection
Eighty days after transplantation complete obstruction of the proximal ureter was observed, complicated by recurrent urinary tract infections. [ncbi.nlm.nih.gov]
urinary tract infection ; urethral discharge and swelling". [4] Risk factors [ edit ] Tobacco smoking is associated with an increased risk of ureteral cancer. [5] Diagnosis [ edit ] Example of ureteral cancer Diagnosis may include a fluorescence in situ [en.wikipedia.org]
She had previously been treated five times by her local general practitioner for recurrent urinary tract infections. The only relevant finding on clinical examination was that of an ill-defined left flank mass with pain on palpation. [liebertpub.com]
The other clinical features included recurrent urinary tract infections, microscopic or gross haematuria as in our reported case 7. Imagings are usually sufficient to make diagnosis of retrocaval ureter. [latunisiemedicale.com]
Regarding risk factors, tobacco smoking was referred by 66% of the patients and 33% had recurrent urinary tract infections or calculi. In one patient there was an hereditary nonpolyposis colorectal cancer syndrome associated (3%). [scielo.br]
- Amyloidosis
’s disease and tuberculosis. 4 Localized amyloidosis is much less common than systemic amyloidosis and occurs in about 10-20% of amyloidosis cases. 3,5 This condition can occur in the lungs, trachea, larynx, tongue, skin, nervous system, gastrointestinal [appliedradiology.com]
Gastrointestinal
- Abdominal Pain
Two of the three patients had abdominal pain, while the other patient had no upper urinary tract symptoms. All patients had excretory urograms that showed delayed or no excretion of contrast medium on the side of obstruction. [jamanetwork.com]
A 79-year-old woman visited her home doctor with the chief complaint of right lower abdominal pain. Abdominal computed tomographic scan (CT) disclosed a tumor measuring about 5 cm in diameter at the right lower quadrant of the abdomen. [ncbi.nlm.nih.gov]
If papillary urothelial carcinoma spreads, it can cause other signs and symptoms, including: loss of appetite swelling in feet pelvic pain abdominal pain tiredness jaundice fever weight loss Bladder cancer overview Bladder cancer can affect any part of [medicalnewstoday.com]
At about 12 months after surgery, the patient had symptoms of frequent urination, urgency, dysuria, and abdominal pain. [dovepress.com]
- Left Flank Pain
This is the case report of a 69‑year‑old male patient who presented with left flank pain and gross hematuria. Following diagnosis of ureteral carcinoma, nephroureterectomy was performed. [spandidos-publications.com]
Case Presentation A 39-year-old, HIV-negative woman presented with a 1-year history of worsening left flank pain, intermittent visible hematuria, and a fullness in the left flank. [liebertpub.com]
The clinical symptom was just left flank pain. Imageology examination showed the hydronephrosis and occupied lesions. Compared with other cases, this one is maybe in an early stage of the tumour. [ncbi.nlm.nih.gov]
Liver, Gall & Pancreas
- Hepatomegaly
[…] outlet obstruction (rare) Suprapubic/rectal/perineal pain Palpable suprapubic mass (advanced cases) Features of uremia Constitutional symptoms : anorexia, weight loss, fatigue Metastatic symptoms Enlarged lymph nodes Liver : right upper quadrant pain, hepatomegaly [amboss.com]
Neurologic
- Asthenia
[…] occurring in 10 percent or more of participants were generally lower with Keytruda as opposed to chemotherapy, respectively, including for fatigue (13.9 percent vs 27.8 percent), nausea (10.9 percent vs 24.3 percent), diarrhea (9.0 percent vs 12.9 percent), asthenia [curetoday.com]
Treatment-related AEs occurring in ≥10% of participants were generally lower with pembrolizumab as opposed to chemotherapy, respectively, including for fatigue (13.9% vs 27.8%), nausea (10.9% vs 24.3%), diarrhea (9.0% vs 12.9%), asthenia (5.6% vs 14.1% [onclive.com]
- Peripheral Neuropathy
neuropathy New York Heart Association class III heart failure In patients with limited renal function, chemotherapy options are limited but can consist of the following: Gemcitabine and carboplatin [34] – Overall response rate, 41.2%; median survival [emedicine.medscape.com]
Pre-existing moderate to severe or severe peripheral neuropathy. [cancertherapyadvisor.com]
Urogenital
- Hematuria
Patients often present with painless gross hematuria or irritative voiding symptoms, although some cases are incidentally detected ( microscopic hematuria on urinalysis ). [amboss.com]
Minhaj Siddiqui, M.D. 1 Citing Article A 69-year-old woman presented to the emergency department with new-onset gross hematuria. Her medical history was notable for 20 pack-years of smoking. [nejm.org]
Gross hematuria was present in all the patients. Three cases had multicentric tumor sites. Three tumors were located in the distal ureter. Four cases showed a non functioning kidney at IVP. [ncbi.nlm.nih.gov]
Hematuria is also a symptom of a metastatic ureteral carcinoma and, in the uncommon case where the ureters are bilaterally affected, patients may present with anuria. [symptoma.com]
- Flank Pain
The second most frequently reported complaint is a flank pain, observed in 1 in 5 patients diagnosed with a ureteral carcinoma. [symptoma.com]
He presented with bilateral flank pain accompanied by nausea, vomiting and oliguria. Bilateral hydronephrosis with upper ureteral filling defects was found on antegrade pyelogram. [ncbi.nlm.nih.gov]
However, in this case, the patient only presented with left-sided flank pain and radiating pain in both the hypogastrium and groin. This clinical feature was similar to the presentation of a ureteric calculus. [bjuinternational.com]
[…] chest pain, pleural effusion Bone: pain in long bones / vertebrae, pathological fractures Carcinoma of the renal pelvis Ureteral carcinoma Painless gross hematuria throughout micturition Flank pain Urethral carcinoma Painless gross hematuria at the beginning [amboss.com]
- Pelvic Pain
If papillary urothelial carcinoma spreads, it can cause other signs and symptoms, including: loss of appetite swelling in feet pelvic pain abdominal pain tiredness jaundice fever weight loss Bladder cancer overview Bladder cancer can affect any part of [medicalnewstoday.com]
Patients present with abnormal vaginal bleeding and can have pelvic pain from local spread of disease or inflammation (, 3 ). [pubs.rsna.org]
Diabetes mellitus Apex 3.3 Partial cystectomy TCC, G2, with squamous and glandular differentiation + osteochondrosarcoma pT2 min N/A 38 (51) F/75 Hematuria, pelvic pain von Recklinghausen disease Left trigone 11 Cystectomy High grade TCC with glandular [onlinelibrary.wiley.com]
- Macroscopic Hematuria
However, on urinalysis, there was macroscopic hematuria and 40–50 white blood cells per high-power field. [spandidos-publications.com]
The vast majority of patients with bladder cancer present with microscopic or macroscopic hematuria (85%). Intermittent painless gross hematuria is the most common presentation. [cancertherapyadvisor.com]
Rhéaume-Lanoie J, Lepanto L, Fradet V et al: Diagnostic performance of ultrasound for macroscopic hematuria in the era of multidetector computed tomography urography. Can Assoc Radiol J 2014; 65: 253. [auanet.org]
Workup
The complete workup involved in diagnosing a ureteral carcinoma consists of blood laboratory testing, imaging modalities and histopathologic assessment of the lesion.
Laboratory testing aims at the evaluation of the general patient status as well as kidney function. A complete blood count and biochemical profile are performed, alongside prothrombin time, international normalized ratio and activated partial thromboplastin time tests, in order to eliminate potential abnormalities in the coagulation process. Urinalysis is a basic initial test since it allows for the confirmation of hematuria and helps to rule out a simple urinary tract infection that could account for the same symptoms elicited by a ureteral carcinoma. As far as imaging is concerned, excretory urography has been presently replaced to a large extent by computerized tomography (CT). A CT urogram that focuses extensively on the excretory stage of urination can illustrate tumors of the ureters, primarily those that are larger than 5 mm in size. CT scans also aid in determining the extent to which a malignancy has spread peripelvically or periureterally, although it contributes very little to the staging process of a transitional cell carcinoma.
Cytology evinces a satisfactory accuracy rate in diagnosing high-grade ureteral carcinomas and more aggressive disease, whereas, in the case of low-grade malignant lesions, the analysis renders cancer-negative results in most of the samples [8]. Low-grade neoplastic lesions are better detected with the use of fluorescence in situ hybridization (FISH) [9] [10]. Ureteroscopy is usually also employed in order to directly visualize a ureteral carcinoma [11].
Urine
- Pyuria
CASE REPORT: 60-year-old phaeodermal female, reported a history of right-side nephritic colic, hyperthermia and pyuria during the past 20 years and had received treatment for urinary infections a number of times. [scielo.br]
Ultrasound
- Enlargement of the Liver
[…] lymph nodes Liver : right upper quadrant pain, hepatomegaly, jaundice, ascites Lungs : dyspnea, cough, chest pain, pleural effusion Bone: pain in long bones / vertebrae, pathological fractures Carcinoma of the renal pelvis Ureteral carcinoma Painless [amboss.com]
Treatment
However, it represents a feasible and effective alternative for the treatment of LG-UC in distal ureter, as long as the oncological and reconstructive principles are respected. [elsevier.es]
CONCLUSION: The conservative treatment of patients presenting with superficial ureteral lesions seems to be a good alternative to radical surgery. [ncbi.nlm.nih.gov]
There are two types of treatment for upper tract urothelial carcinomas: The tumor location and grade will indicate the type of treatment needed. [saintjohnscancer.org]
Treatment options can include a combination of the following: surgery chemotherapy biologic therapy radiation Surgery Surgical treatment options for papillary urothelial carcinoma vary. [medicalnewstoday.com]
Radiographic features of the other imaging modalities may be nonspecific, but it is important for clinicians and radiologists to be cognizant of this disease so proper management and treatment may be provided. [appliedradiology.com]
Prognosis
Due to the way this type of cancer forms, it tends to be fairly easy to treat and has a good prognosis. [medicalnewstoday.com]
Prognosis appears to be determined by the bladder tumor. [ncbi.nlm.nih.gov]
Prognosis depends on the stage of the tumor (see staging of TTCs of the ureter ) and histological grade (see grading of TCCs ) has little influence 3. Metastases are most frequently to liver, bone, and lung 4. Promoted articles (advertising) [radiopaedia.org]
Etiology
Frequency, etiology, clinical, diagnostic findings and surgical therapy as well have all been taken into account, based on the literature. Histopathologic aspects, grade and stage, are considered too. [ncbi.nlm.nih.gov]
Critical evaluation of environmental exposure agents suspected in the etiology of Balkan endemic nephropathy. Int J Occup Environ Health. 2006;12(4):369–76. PubMed 6. Grollman AP, Shibutani S, Moriya M, et al. [springermedizin.at]
[…] urothelial carcinoma is not synonymous with WHO 1973 grade 1 Epidemiology Incidence: 5 per 100,000 individuals per year Mean age 70 years 75% male Sites Commonly on posterior bladder wall Lateral wall close to ureteral orifices is another common site Etiology [pathologyoutlines.com]
Cystoscopy and biopsy : direct visualization of urethral and bladder mucosa with possible simultaneous biopsies or therapeutic resections Ultrasound (kidney, ureter, bladder ) : if CT is contraindicated (e.g., pregnant women), or if etiology of hematuria [amboss.com]
Epidemiology
Epidemiological aspects of Balkan endemic nephropathy in a typical focus in Yugoslavia. IARC Sci Publ. 1991;115:5–10. PubMed 34. Bukvic´ D, Jankovic´ S, Markovic´-Denic´ Lj. [springermedizin.at]
Epidemiology Urothelial cancer ; is the most common tumor of the urinary tract. [amboss.com]
Epidemiology of transitional cell carcinomas of the renal pelvis are similar to those of the rest of the urinary tract: please refer to TCCs of urinary tract for further details. [radiopaedia.org]
Pathophysiology
Conclusion: The pathogenesis and pathophysiology of PTTM remains obscure with no effective management available. [karger.com]
Pathophysiology Types of upper urinary tract tumors Transitional cell carcinoma (TCC) is the most common histology observed, accounting for greater than 95% of upper urinary tract urothelial tumors. TCCs are strongly associated with smoking. [emedicine.medscape.com]
Pathophysiology Ta and Tis have distinct molecular pathways and clinical course. Two distinct molecular pathways of oncogenesis and tumor progression are recognized in urothelial carcinoma of the bladder (Ta). [cancertherapyadvisor.com]
Prevention
[…] strategies Discussion of the benefits and limitations of genetic testing Genetic testing, if appropriate Opportunity to participate in research studies focused on cancer risk and prevention Make an Appointment Please call Laura Gross, BA (Program Manager [hospitals.jefferson.edu]
Causes, Risk Factors, and Prevention Learn about the risk factors for bladder cancer and what you might be able to do to help lower your risk. Early Detection, Diagnosis, and Staging Know the signs and symptoms of bladder cancer. [cancer.org]
The drugs kill cancer cells, preventing them from dividing and spreading further. Read more Meet our doctors Everything we do is focused on designing better care for our patients. [genesiscare.co.uk]
Immunotherapy in the form of Bacillus Calmette-Guerin (BCG) instillation can also be used to treat and prevent the reappearance of superficial tumors related to cancer of the bladder. [roboticoncology.com]
References
- Ito Y, Kikuchi E, Tanaka N, et al. Preoperative hydronephrosis grade independently predicts worse pathological outcomes in patients undergoing nephroureterectomy for upper tract urothelial carcinoma. J Urol. 2011;185:1621–1626.
- Jang H, Yuk SM, Kim JO, Han DS. A rare case of primary malignant small cell carcinoma combined with urothelial cell carcinoma in the ureter. World J Surg Oncol. 2013; 11: 181.
- Xiong X, Jia L, Wang J. Primary adenocarcinoma of the renal pelvis, ureter and the urinary bladder: A case report and review of the literature. Oncol Lett. 2016 Mar; 11(3): 1811–1814.
- Cohen WM, Freed SZ, Hasson J. Metastatic cancer to the ureter: a review of the literature and case presentations. J Urol. 1974;112:188–189.
- Winalski CS, Lipman JC, Tumeh SS. Ureteral neoplasms. Radiographics. 1990;10:271–283.
- Richie JP, Withers G, Ehrlich RM. Ureteral obstruction secondary to metastatic tumors. Surg Gynecol Obstet. 1979;148:355–357.
- Puech JL, Song MY, Joffre F, et al. Ureteral metastases- computed tomographic findings. Eur J Radiol. 1987;7:103–106.
- Brien JC, Shariat SF, Herman MP, Ng CK, Scherr DS, Scoll B. Preoperative hydronephrosis, ureteroscopic biopsy grade and urinary cytology can improve prediction of advanced upper tract urothelial carcinoma. J Urol. 2010 Jul. 184(1):69-73.
- Luo B, Li W, Deng CH, Zheng FF, Sun XZ, Wang DH. Utility of fluorescence in situ hybridization in the diagnosis of upper urinary tract urothelial carcinoma. Cancer Genet Cytogenet. 2009 Mar. 189(2):93-7.
- Mian C, Mazzoleni G, Vikoler S, Martini T, Knuchel-Clark R, Zaak D. Fluorescence in situ hybridisation in the diagnosis of upper urinary tract tumours. Eur Urol. 2010 Aug. 58(2):288-92.
- Sarosdy MF, Kahn PR, Ziffer MD, Love WR, Barkin J, Abara EO. Use of a multitarget fluorescence in situ hybridization assay to diagnose bladder cancer in patients with hematuria. J Urol. 2006 Jul. 176(1):44-7.