Welcome to the Rare Classroom, a new series from Patient Worthy. Rare Classroom is designed for the curious reader who wants to get informed on some of the rarest, most mysterious diseases and conditions. There are thousands of rare diseases out there, but only a very small number of them have viable treatments and regularly make the news. This series is an opportunity to learn the basics about some of the diseases that almost no one hears much about or that we otherwise haven’t been able to report on very often.

Eyes front and ears open. Class is now in session.

The rare disease that we will be learning about today is:

Urothelial Cancer

Also called transitional cell carcinoma or urothelial carcinoma.

What is Urothelial Cancer?

• Urothelial cancer is a type of cancer that occurs in the urinary system
• The most common form is urothelial carcinoma, and it is the most common cancer of the bladder, ureter, urachus, and urethra
• This cancer affects a tissue called the transitional epithelium
• Urothelial cancer is diagnosed with imaging of the urinary tract and analysis of the urine itself

How Do You Get It?

• A number of risk factors for urothelial cancer have been identified, which include:
  • Family history
  • Tobacco smoking
  • Exposure to certain chemicals
  • Frequent bladder infections
  • History of radiation therapy
  • Being male (risk of urothelial cancer is four times higher in men)
• Age of onset is usually between 65 and 84 years

What Are the Symptoms?

• Signs and symptoms include:
  • Blood in the urine
  • Frequent urination
  • Pain during urination
  • Urge to urinate without being able to
  • Flank pain (metastatic urothelial cancer)
  • Pelvic or bone pain (metastatic urothelial cancer)

How Is It Treated?

• Early-stage disease may be treated with Bacille Calmette-Guerin, which is infused into the bladder
  • Surgery may also be attempted, such as cysto-prostatectomy
• Metastatic:
  • Cisplatin-based chemotherapy is the standard treatment for metastatic urothelial cancer.
    • One example is a four-part combination regimen of methotrexate, vinblastine, adriamycin, and cisplatin
  • Second-line immunotherapy or chemotherapy may be used if the cancer returns or progresses
  • Atezolizumab was given FDA accelerated approval in May 2016
  • Sacituzumab govitecan was given FDA accelerated approval in April 2021
• Quality of life and outcomes vary considerably in urothelial cancer. Five-year survival rate is around 77% in the US.

Where Can I Learn More???

• Check out our cornerstone on this disease here.
• Learn more about this disease from the Bladder Cancer Advocacy Network.