Disease Description (Causes · Pathophysiology · Main Symptoms)
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Definition summary
- Renal pelvis cancer (upper urinary tract urothelial carcinoma) is a cancer that arises from the mucosa (mainly transitional epithelium/urothelium) of the renal pelvis (renal calyces) and ureter. Lesions may be multifocal and recurrent, and there is a high risk of reciprocal recurrence (or simultaneous occurrence) with bladder cancer.
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Causes and risk factors
- No single definitive cause has been identified. Known risk factors include smoking, occupational exposure to carcinogens (e.g., past exposure to dyes and organic chemicals), long-term use of certain analgesics (e.g., phenacetin), some anticancer drugs such as cyclophosphamide, chronic inflammation, and urinary tract stones.
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Pathophysiology summary
- It arises from the epithelium (transitional epithelium) and may progress as superficial disease (mucosa and submucosa) or invasive disease (muscular layer, renal parenchyma, surrounding tissues, and lymph nodes). Because the ureter has a thin wall, the cancer may spread rapidly; obstruction of the ureter by the tumor can cause hydronephrosis (dilation due to urine accumulation) and decreased kidney function. Superficial disease has a high rate of recurrence in the bladder (approximately 30–40%).
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Main symptoms
- Visible hematuria (the most common symptom), flank or back pain (when there is ureteral obstruction or invasion of surrounding tissues), detection during a health checkup despite no symptoms, and a nonfunctioning kidney due to chronic obstruction (decreased kidney function without symptoms), among others.
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Main diagnostic tests (summary)
- Urine cytology (cancer cells in urine), cystoscopy (to rule out bladder tumors and observe the ureteral orifice), abdominal ultrasonography (hydronephrosis and masses), CT (evaluation of the urinary tract and metastasis), retrograde pyelography (RGP), etc.
Treatment Methods and Precautions
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Localized disease (no metastasis)
- Standard surgery: Radical nephroureterectomy + removal of the bladder wall around the ureteral orifice (bladder cuff) — the standard treatment for invasive lesions of the upper urinary tract.
- Kidney function-preserving treatment: For solitary, low-grade superficial lesions, endoscopic resection/excision and laser ablation (transurethral surgery) or partial resection (removal of part of the ureter) may be considered in selected patients.
- Radiation therapy: Transitional cell carcinoma is generally not highly radiosensitive, so radiation is not a standard treatment (it may be considered for local palliation or when surgery is not possible).
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Metastatic/unresectable disease
- Systemic chemotherapy: Cisplatin-based combination therapy (e.g., gemcitabine + cisplatin) is the standard first-line treatment. If cisplatin cannot be used because of kidney function or other factors, a carboplatin-based regimen or immune checkpoint inhibitors may be considered, depending on guidelines and PD-L1 status.
- Immunotherapy and targeted therapy: In metastatic urothelial carcinoma, PD-1/PD-L1 inhibitors (e.g., pembrolizumab and atezolizumab) and targeted therapy (e.g., erdafitinib) for patients with specific gene mutations such as FGFR3 may be used in some cases — genetic testing and confirmation of indications are required.
- Adjuvant (after surgery) and neoadjuvant (before surgery) chemotherapy: May be considered for high-risk invasive lesions, based on individualized assessment.
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Complications and medication adverse effects (main items)
- Surgery: Most people can carry out daily activities after removal of one kidney (the need for dialysis is uncommon). General surgical complications, such as bleeding and infection, may occur.
- Anticancer drugs: Bone marrow suppression (decreased white blood cells and platelets), worsening kidney function, nausea and vomiting, decreased appetite, hair loss, etc. Immunotherapy may cause immune-related adverse effects involving the lungs, liver, endocrine organs, and other organs.
- Radiation: Local skin or mucosal symptoms or damage to surrounding tissues (limited when used).
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Follow-up and recurrence management (precautions)
- Because the risk of bladder recurrence is high, long-term follow-up (usually lifelong) after surgery is required with periodic cystoscopy and urine tests (urine cytology), as well as upper urinary tract imaging (CT/ultrasonography).
- Smokers are strongly advised to quit smoking. Avoid nephrotoxic drugs, and regular kidney function tests (serum creatinine) are recommended.
Checklist Before an Outpatient Visit (Items to Bring · Test Information · Sample Questions)
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Items recommended to bring
- A complete list of current medications and supplements, including doses and administration times
- Copies of records of previous surgeries and cancer treatments, as well as pathology and test results (biopsy and urine cytology), if possible
- Recent test results (blood tests, kidney function/creatinine, urinalysis) and images/reports from imaging studies (CT and ultrasonography)
- A record of when symptoms began and how often they occur (frequency and color of hematuria; location, severity, and duration of pain)
- Allergies (including medications and contrast agents) and a history of adverse effects from previous anticancer drugs
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Items useful to organize in advance for the outpatient visit (briefly)
- Recent weight changes, history of fever or infection, changes in urination habits, and smoking and occupational history (whether there has been exposure to chemicals)
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Situations that should be reported to the doctor immediately during the outpatient visit
- Sudden high fever (possible immunocompromised state), severe flank pain, or persistent bleeding and dizziness (possible anemia)
General Sample Questions to Ask the Doctor
- "What are my current stage and grade? What is the prognosis?"
- "What are the standard treatment options in my case (surgery, kidney-preserving treatment, and chemotherapy), and what are their advantages and disadvantages?"
- "What is the risk of recurrence after surgery, and how will follow-up testing be performed (frequency and methods)?"
- "What are the major adverse effects of the anticancer drugs that will be used, and how can they be prevented and managed?"
- "If I cannot receive cisplatin because of my kidney function, what alternatives are available?"
Examples of Disease-Specific Questions
- "One of my kidneys has reduced function (or I have a solitary kidney). Are there treatment options that can preserve my kidney? How high is the risk of recurrence if it is preserved?"
- "I have heard that cancer is likely to recur in the bladder. What bladder prevention and surveillance strategies are used after surgery (nephroureterectomy)? (For example, how often is cystoscopy performed, and could intravesical treatment be needed?)"