Biliary Tract Cancer

Updated: September 26, 2024

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Background

Biliary Tract Cancer is a group of rare and aggressive malignancies that arise from the bile ducts, gallbladder, and ampulla of Vater.

Gallbladder cancer is considered as the most common biliary tract and third gastrointestinal tract cancer.

Tumors spread through blood vessels, lymph nodes, nerves, and ducts to liver/organs.

These cancers are divided into three types:

Cholangiocarcinoma: It is sub-divided as:

Intrahepatic Cholangiocarcinoma: It occurs in the bile ducts within the liver

Perihilar Cholangiocarcinoma: It occur at the junction of the right and left hepatic bile ducts

Distal Cholangiocarcinoma: It occurs in the bile ducts outside the liver near the small intestine

Gallbladder Cancer

Ampullary Cancer

Gallbladder cancer arises from the gallbladder’s epithelial cells. It occurs more in women as compared to men.

Ampullary cancer occurs where the bile duct and pancreatic duct meet and empty into the small intestine at the ampulla of Vater.

Epidemiology

Cholangiocarcinoma incidence rates range from 0.72 to 1.62 per 100000 in the U.S.

Northeast Thailand has the highest disease incidence rate of over 80 per 100000 individuals.

Studies suggests a rise in intrahepatic cholangiocarcinoma incidence due to chronic inflammation and irritation of the biliary tract epithelium over decades.

Incidence of primary sclerosing cholangitis increases with age and is more common in men due to high prevalence.

Anatomy

Pathophysiology

Chronic irritation and inflammation cause similar changes to the biliary tract epithelium as other GI cancers.

Research indicates that 20% of intrahepatic bile duct tumors have mutations in IDH1 and IDH2 genes.

While 13% to 17% cases exhibit fibroblast growth factor receptor gene fusion or translocation.

P53 mutation is present in 40% of perihilar and distal cholangiocarcinoma and 25% of intrahepatic cholangiocarcinoma.

Etiology

The causes of biliary tract cancer are:

Chronic Inflammation and Injury

Genetic Mutations

Biliary Tract Abnormalities

Viral and Parasitic Infections

Environmental and Chemical Exposures

Obesity and Metabolic Syndrome

Genetics

Prognostic Factors

Biliary tract cancer has poor prognosis with survival less than 2 years and less than 10% survival rate.

Chemotherapeutic agents treat unresectable tumors, but overall median survival is only one year.

Surgery is potential cure for early cancer and post-surgery survival factors.

Undifferentiated tumors show aggressive behavior and worse outcomes.

Clinical History

Collect details including initial symptoms, progression of symptoms, and medical history to understand clinical history of patient.

Physical Examination

Abdominal examination

Full body assessment

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Chronic symptoms are:

Jaundice

Abdominal Pain

Weight Loss and Fatigue

Nausea and Anorexia

Acute symptoms are:

Biliary Obstruction

Severe Abdominal Pain

Pruritus

Severe right upper quadrant pain

Acute Biliary Colic

Severe abdominal distention

Differential Diagnoses

Hepatocellular carcinoma

Gallbladder adenomas

Metastatic liver cancer

Gallbladder adenomyomatosis

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Surgical resection is the sole cure for localized bile duct tumors. Evaluate the patient physically and radiologically before surgery.

Surgical exploration via laparoscopy may be needed for staging tumor before resecting cholangiocarcinoma.

Post-surgery monitoring for biliary tract issues using post-treatment CT imaging

Late-stage diagnosis in metastatic or recurrent bile duct cancers or unfit for surgery.

Chemotherapy for advanced or unresectable tumors benefits from gemcitabine alone or combined with cisplatin based on clinical trials.

Recent studies recommend targeted therapies for patients with unresectable tumors due to emerging data on molecular treatments.

Cholangiocarcinoma can have fibroblast growth factor receptor gene fusion. Inhibitors may improve survival in unresectable tumors.

Bevacizumab tested with GEMOX and erlotinib in combination shows promising anti-cancer potential.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-non-pharmacological-approach-for-biliary-tract-cancer

Moving the bed closer to a bathroom or using bedside commodes can prevent falls and reduce effort for patients.

Proper nutrition is of great importance for biliary tract cancer patients, particularly those who are undergoing the treatment.

Alternative pain management approaches like relaxation therapy, an imagery guide, acupuncture, and massage can help sufferers to feel less painful and injured.

Physical activity and exercise can be a good way to improve the strength, endurance and overall wellness of the patients.

Proper awareness about biliary tract cancer should be provided and its related causes with management strategies.

Appointments with an oncologist and preventing recurrence of disorder is an ongoing life-long effort.

Use of Chemotherapy

Gemcitabine/Cisplatin:

Gemcitabine interferes with DNA replication to cause cancer cell death.

While cisplatin induces DNA cross-linking to results in apoptosis.

Use of Targeted Therapy

Infigratinib:

It is an oral FGFR inhibitor used in patients with FGFR2 gene fusions.

Pembrolizumab:

It blocks PD-1 inhibitor that enhances the immune system ability to attack cancer cells.

use-of-intervention-with-a-procedure-in-treating-biliary-tract-cancer

Biliary drainage and stenting are the most commonly used interventions to relieve obstruction.

In patients with unresectable intrahepatic cholangiocarcinoma, tumor ablation techniques may be used to control tumor growth.

use-of-phases-in-managing-biliary-tract-cancer

In initial treatment phase, evaluation of staging tumor and diagnostic test to confirm diagnosis.

Pharmacologic therapy is effective in the treatment phase as it includes use of targeted and chemo therapies.

In supportive care and management phase, patients should receive required attention such as lifestyle modification and surgical intervention.

The regular follow-up visits with the oncologist are scheduled to check the improvement of patients along with treatment response.

Medication

 

pembrolizumab / Berahyaluronidase 

Administer dose of 395 mg/4,800 units through subcutaneous route every three weeks Administer prior to chemotherapy when given on the same day



 
 

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Biliary Tract Cancer

Updated : September 26, 2024

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Biliary Tract Cancer is a group of rare and aggressive malignancies that arise from the bile ducts, gallbladder, and ampulla of Vater.

Gallbladder cancer is considered as the most common biliary tract and third gastrointestinal tract cancer.

Tumors spread through blood vessels, lymph nodes, nerves, and ducts to liver/organs.

These cancers are divided into three types:

Cholangiocarcinoma: It is sub-divided as:

Intrahepatic Cholangiocarcinoma: It occurs in the bile ducts within the liver

Perihilar Cholangiocarcinoma: It occur at the junction of the right and left hepatic bile ducts

Distal Cholangiocarcinoma: It occurs in the bile ducts outside the liver near the small intestine

Gallbladder Cancer

Ampullary Cancer

Gallbladder cancer arises from the gallbladder’s epithelial cells. It occurs more in women as compared to men.

Ampullary cancer occurs where the bile duct and pancreatic duct meet and empty into the small intestine at the ampulla of Vater.

Cholangiocarcinoma incidence rates range from 0.72 to 1.62 per 100000 in the U.S.

Northeast Thailand has the highest disease incidence rate of over 80 per 100000 individuals.

Studies suggests a rise in intrahepatic cholangiocarcinoma incidence due to chronic inflammation and irritation of the biliary tract epithelium over decades.

Incidence of primary sclerosing cholangitis increases with age and is more common in men due to high prevalence.

Chronic irritation and inflammation cause similar changes to the biliary tract epithelium as other GI cancers.

Research indicates that 20% of intrahepatic bile duct tumors have mutations in IDH1 and IDH2 genes.

While 13% to 17% cases exhibit fibroblast growth factor receptor gene fusion or translocation.

P53 mutation is present in 40% of perihilar and distal cholangiocarcinoma and 25% of intrahepatic cholangiocarcinoma.

The causes of biliary tract cancer are:

Chronic Inflammation and Injury

Genetic Mutations

Biliary Tract Abnormalities

Viral and Parasitic Infections

Environmental and Chemical Exposures

Obesity and Metabolic Syndrome

Biliary tract cancer has poor prognosis with survival less than 2 years and less than 10% survival rate.

Chemotherapeutic agents treat unresectable tumors, but overall median survival is only one year.

Surgery is potential cure for early cancer and post-surgery survival factors.

Undifferentiated tumors show aggressive behavior and worse outcomes.

Collect details including initial symptoms, progression of symptoms, and medical history to understand clinical history of patient.

Abdominal examination

Full body assessment

Chronic symptoms are:

Jaundice

Abdominal Pain

Weight Loss and Fatigue

Nausea and Anorexia

Acute symptoms are:

Biliary Obstruction

Severe Abdominal Pain

Pruritus

Severe right upper quadrant pain

Acute Biliary Colic

Severe abdominal distention

Hepatocellular carcinoma

Gallbladder adenomas

Metastatic liver cancer

Gallbladder adenomyomatosis

Surgical resection is the sole cure for localized bile duct tumors. Evaluate the patient physically and radiologically before surgery.

Surgical exploration via laparoscopy may be needed for staging tumor before resecting cholangiocarcinoma.

Post-surgery monitoring for biliary tract issues using post-treatment CT imaging

Late-stage diagnosis in metastatic or recurrent bile duct cancers or unfit for surgery.

Chemotherapy for advanced or unresectable tumors benefits from gemcitabine alone or combined with cisplatin based on clinical trials.

Recent studies recommend targeted therapies for patients with unresectable tumors due to emerging data on molecular treatments.

Cholangiocarcinoma can have fibroblast growth factor receptor gene fusion. Inhibitors may improve survival in unresectable tumors.

Bevacizumab tested with GEMOX and erlotinib in combination shows promising anti-cancer potential.

Oncology, Medical

Moving the bed closer to a bathroom or using bedside commodes can prevent falls and reduce effort for patients.

Proper nutrition is of great importance for biliary tract cancer patients, particularly those who are undergoing the treatment.

Alternative pain management approaches like relaxation therapy, an imagery guide, acupuncture, and massage can help sufferers to feel less painful and injured.

Physical activity and exercise can be a good way to improve the strength, endurance and overall wellness of the patients.

Proper awareness about biliary tract cancer should be provided and its related causes with management strategies.

Appointments with an oncologist and preventing recurrence of disorder is an ongoing life-long effort.

Oncology, Medical

Gemcitabine/Cisplatin:

Gemcitabine interferes with DNA replication to cause cancer cell death.

While cisplatin induces DNA cross-linking to results in apoptosis.

Oncology, Medical

Infigratinib:

It is an oral FGFR inhibitor used in patients with FGFR2 gene fusions.

Pembrolizumab:

It blocks PD-1 inhibitor that enhances the immune system ability to attack cancer cells.

Oncology, Medical

Biliary drainage and stenting are the most commonly used interventions to relieve obstruction.

In patients with unresectable intrahepatic cholangiocarcinoma, tumor ablation techniques may be used to control tumor growth.

Oncology, Medical

In initial treatment phase, evaluation of staging tumor and diagnostic test to confirm diagnosis.

Pharmacologic therapy is effective in the treatment phase as it includes use of targeted and chemo therapies.

In supportive care and management phase, patients should receive required attention such as lifestyle modification and surgical intervention.

The regular follow-up visits with the oncologist are scheduled to check the improvement of patients along with treatment response.

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