Holly Stachler Stachler1

Professor Kretzer

English 1201

14 November 2021
What is Cholangiocarcinoma?

Cholangiocarcinoma is a rare type of cancer that begins in the bile ducts, and it is also

known as bile duct cancer. Bile ducts are tubes that connect the liver and gallbladder to the small

intestine. These tubes carry bile, which is a fluid that helps the body digest the fats in food

(“Cholangiocarcinoma: Medlineplus Genetics”,2020). Studies show that a combination of

genetic, environmental, and lifestyle factors influence whether a person will develop

cholangiocarcinoma (“Cholangiocarcinoma: Medlineplus Genetics”, 2020). The causes,

symptoms, and treatments are continually being researched by medical professionals.

Cancers occur when there is a mutation buildup in critical genes that causes cells to grow

and divide uncontrollably forming a tumor (Cholangiocarcinoma: Medlineplus Genetics, 2020).

In cases of cholangiocarcinoma, these genetic changes are acquired during a person’s lifetime

and are only present in the bile ducts (Cholangiocarcinoma: Medlineplus Genetics, 2020).

Somatic mutations can occur in genes that are tumor suppressors, which are genes that help

control the growth and division of cells. Other genes associated with cholangiocarcinoma are

oncogenes. When oncogenes are activated irregularly, they can cause normal cells to be

cancerous (“Cholangiocarcinoma: Medlineplus Genetics”, 2020).

The different types of cholangiocarcinoma are identified by their location relative to the

liver (“Cholangiocarcinoma: Medlineplus Genetics”, 2020). Intrahepatic cholangiocarcinoma

begins in the bile ducts within the liver, this is the least common form of this disease (National

Cancer Institute). Hilar cholangiocarcinoma grows in the bile ducts right outside of the liver.

This is the most common form of this disease. The remaining types of bile duct cancer are called
Holly Stachler Stachler2

Professor Kretzer

English 1201

14 November 2021
distal cholangiocarcinomas that begin in the parts of the bile ducts near the small intestine

(National Cancer Institute). Sometimes hilar cholangiocarcinoma and distal cholangiocarcinoma

are grouped together and called extrahepatic cholangiocarcinoma (National Cancer Institute).

There are many risk factors for developing cholangiocarcinoma. Some risk factors

include: primary sclerosing cholangitis, chronic ulcerative colitis, cysts in the bile ducts,

cirrhosis of the liver, Hepatitis B or C virus, diabetes, obesity, genetic factors, and past infection

with liver flukes (Veterans Health Administration, 2017). Other factors that influence the

development of cholangiocarcinoma are inflammatory bowel disease, exposure to certain

chemical toxins used in manufacturing, and lifestyle factors such as smoking, and alcohol use

(“Cholangiocarcinoma: Medlineplus Genetics”, 2020).

Cholangiocarcinoma doesn't cause symptoms in the early stages so it is usually not

diagnosed until it has spread to other tissues in the body (“Cholangiocarcinoma: Medlineplus

Genetics”, 2020). Symptoms arise when the bile ducts get blocked by the tumor. Symptoms of

bile duct cancer include jaundice, fatigue, itching, dark-colored urine, loss of appetite, weight

loss, and stomach pain (“Cholangiocarcinoma: Medlineplus Genetics”). Health care providers

use medical history, physical examination, blood tests, medical imaging, and consultation with

specialists to diagnose bile duct cancer (Veterans Health Administration, 2017).

Recurrent bile duct cancer has poor prognosis, and no optimal treatment procedure has

been established (Koh, 2021). Prognosis depends on where the tumor is, if the cancer has spread,

and how much of the tumor was taken out during surgery (National Cancer Institute). Surgical

resection is the only curative treatment option for locoregional extrahepatic bile duct cancer, but
Holly Stachler Stachler3

Professor Kretzer

English 1201

14 November 2021
surgical candidates are limited (Koh, 2021). More than half of patients with bile duct cancer

develop recurrence even after curative resection. Studies on this treatment have shown a median

survival duration of 19 months and a 3 year survival rate of 32% which is significantly higher

than untreated patients (Koh, 2021). For those unsuitable for surgical resection, other treatments

include radiation therapy, chemotherapy, targeted therapy, and immunotherapy (National Cancer

Institute). The first FDA approved targeted treatment for adults with certain types of previously

treated, advanced cholangiocarcinoma is Pemazyre (Office of the Commissioner, 2020). FGFR2

fusions have been found in the tumors of 9% to 14% of patients with cholangiocarcinoma.

Pemazyre is a tablet that works by blocking FGFR2 in tumor cells to prevent them from growing

or spreading (Office of the Commissioner, 2020).

Cholangiocarcinoma is rare and affects about 8,000 people per year in the United States

(National Cancer Institute). Individuals with this disease can survive for several months or years

after diagnosis depending on the location and advancement of the cancer (“Cholangiocarcinoma:

Medlineplus Genetics”, 2020). Options for treatment of this disease are very slim, and even with

treatment there is poor prognosis. Overall, these sources led me to conclude that

cholangiocarcinoma is an extremely tragic disease with a small chance of patient survival.

Holly Stachler Stachler4

Professor Kretzer

English 1201

14 November 2021
Works Cited

“Cholangiocarcinoma: Medlineplus Genetics.” MedlinePlus, U.S. National Library of

Medicine, 18 Aug. 2020,

https://medlineplus.gov/genetics/condition/cholangiocarcinoma/. Accessed 07 Nov. 2021.

Commissioner, Office of the. “FDA Approves First Targeted Treatment for Patients with

Cholangiocarcinoma, a Cancer of Bile Ducts.” U.S. Food and Drug Administration,

FDA, 17 Apr. 2020, https://www.fda.gov/news-events/press-announcements/fda-

approves-first-targeted-treatment-patients-cholangiocarcinoma-cancer-bile-ducts.

Accessed 07 Nov. 2021.

“Comprehensive Cancer Information.” Edited by Patricia A. Ganz, National Cancer

Institute, Oxford University Press, https://www.cancer.gov/. Accessed 07 Nov. 2021.

Koh, Minji, et al. “Radiation Therapy for Recurrent Extrahepatic Bile Duct Cancer.”

PLoS ONE, vol. 16, no. 6, June 2021, pp. 1–12. EBSCOhost,

doi:10.1371/journal.pone.0253285. Accessed 07 Nov. 2021.

Veterans Health Administration. “Bile Duct Cancer (Cholangiocarcinoma) and Liver

Fluke Infection.” US Department of Veterans Affairs, 17 Mar. 2017,

https://www.publichealth.va.gov/exposures/infectious-diseases/cholangiocarcinoma.asp.

Accessed 07 Nov. 2021