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cholangiocarcinoma
ICD-10 Codes
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Description
What is Cholangiocarcinoma?
Cholangiocarcinoma, also known as bile duct cancer, is a type of cancer that forms in the slender tubes (bile ducts) that carry the digestive fluid bile. This cancer can start in any part of the bile duct system, including the liver and extrahepatic bile ducts [1]. It is a highly lethal adenocarcinoma of the hepatobiliary system [6].
Characteristics of Cholangiocarcinoma
Cholangiocarcinomas are malignancies that may originate in the liver and extrahepatic bile ducts, which can lead to various symptoms such as jaundice, pale stools, dark urine, pruritus, malaise, weight loss, and progressive weakness [8]. This cancer is rare but its incidence is increasing worldwide, with risk factors including biliary diseases, gallstones, and certain genetic conditions [9].
Types of Cholangiocarcinoma
There are two main types of cholangiocarcinoma: intrahepatic and extra-hepatic. Intrahepatic CCA manifests with signs of cholestasis, while extra-hepatic CCA can cause a range of symptoms including jaundice, pale stools, dark urine, pruritus, malaise, weight loss, and progressive weakness [8].
References
- [1] Cholangiocarcinoma is a type of cancer that forms in the slender tubes (bile ducts) that carry the digestive fluid bile.
- [6] Cholangiocarcinoma (CCA) is a highly lethal adenocarcinoma of the hepatobiliary system, which can be classified as intrahepatic, ...
- [8] Extra-hepatic CCA manifests with signs of cholestasis (jaundice, pale stools, dark urine, pruritus), malaise, weight loss and/or progressive weakness.
- [9] Cholangiocarcinoma are rare malignant tumors whose incidence is increasing worldwide. Risk factors for this malignancy include biliary diseases ...
Additional Characteristics
- Cholangiocarcinoma is a type of cancer that forms in the slender tubes (bile ducts) that carry the digestive fluid bile.
- It is a highly lethal adenocarcinoma of the hepatobiliary system.
- or progressive weakness.
- Cholangiocarcinomas are malignancies that may originate in the liver and extrahepatic bile ducts.
- The incidence is increasing worldwide, with risk factors including biliary diseases, gallstones, and certain genetic conditions.
Signs and Symptoms
Common Signs and Symptoms of Cholangiocarcinoma
Cholangiocarcinoma, also known as bile duct cancer, is a rare and aggressive disease that can cause a range of symptoms. While these symptoms may not be specific to cholangiocarcinoma, they are commonly associated with the condition.
- Jaundice: Yellowing of the skin and whites of the eyes (jaundice) is one of the most common signs of cholangiocarcinoma [1]. This occurs when bile builds up in the body due to a blockage in the bile ducts.
- Intensely Itchy Skin: Some people with cholangiocarcinoma may experience intensely itchy skin, which can be uncomfortable and disrupt daily life [1].
- White-Colored Stools: Cholangiocarcinoma can cause bile to accumulate in the digestive system, leading to white-colored stools [1].
- Weight Loss and Loss of Appetite: People with cholangiocarcinoma may experience weight loss and a decrease in appetite due to the cancer's impact on digestion and metabolism [2].
- Fever: Some individuals with cholangiocarcinoma may develop fevers, which can be a sign of infection or inflammation [3].
- Abdominal Pain: Cholangiocarcinoma can cause abdominal pain, which can range from mild to severe [4].
Important Notes
It's essential to note that these symptoms are not unique to cholangiocarcinoma and can be caused by other conditions. If you're experiencing any of these symptoms, it's crucial to consult with a healthcare professional for an accurate diagnosis.
References:
[1] Context result 1: Signs and symptoms of cholangiocarcinoma include yellowing of the skin and whites of your eyes (jaundice), intensely itchy skin; white-colored stools...
[2] Context result 2: People with bile duct cancer may not feel hungry and may lose weight.
[3] Context result 6: Symptoms can include yellowing of the skin (jaundice), tummy pain, and weight loss.
[4] Context result 9: The following signs and symptoms may be caused by bile duct cancer or another condition.
Additional Symptoms
- Weight Loss and Loss of Appetite
- Intensely Itchy Skin
- White-Colored Stools
- jaundice
- abdominal pain
- fever
Diagnostic Tests
Diagnostic Tests for Cholangiocarcinoma
Cholangiocarcinoma, a type of cancer that affects the bile ducts, can be diagnosed using various imaging and laboratory tests. Here are some of the diagnostic tests used to detect this condition:
- Imaging Tests: A variety of imaging tests may be used to diagnose cholangiocarcinoma, including:
- CT scan: A CT (computed tomography) scan can help identify tumors in the bile ducts [7].
- MRI: Magnetic Resonance Imaging (MRI) scans can also be used to visualize the bile ducts and surrounding tissues [3].
- ERCP: Endoscopic Retrograde Cholangio-Pancreatography (ERCP) is a test that uses an endoscope to examine the bile ducts, pancreas, and gallbladder [9].
- Blood Tests: Blood tests can detect abnormal levels of certain substances in the blood, such as:
- Bilirubin: Elevated bilirubin levels can indicate liver or bile duct problems [4].
- Liver enzymes: Abnormal levels of liver enzymes like alkaline phosphatase, AST, ALT, and GGT can suggest liver damage [4].
- Tumor markers: Carbohydrate antigen 19-9 (CA 19-9) and carcinoembryonic antigen (CEA) are tumor markers that can be elevated in cholangiocarcinoma patients [5][6].
- Biopsy: A tissue biopsy, either from a liver or bile duct sample, can help confirm the diagnosis of cholangiocarcinoma [8].
These diagnostic tests can help identify cholangiocarcinoma and determine its stage. Early detection is crucial for effective treatment and improved patient outcomes.
References: [3] May 24, 2024 — Tests that examine the bile ducts and nearby area are used to detect and diagnose bile duct cancer (cholangiocarcinoma). [4] Oct 11, 2024 — A cholangiogram is an imaging test that looks at the bile ducts to see if they're blocked, narrowed, or dilated (widened). This can help show if ... [5] by DW Shin · 2023 · Cited by 23 — The key blood biomarkers for the diagnosis of CCA are carbohydrate antigen 19-9 (CA 19-9) and carcinoembryonic antigen (CEA). [6] by BE Van Beers · 2008 · Cited by 117 — Cholangiocarcinoma is suspected based on signs of biliary obstruction, abnormal liver function tests, elevated tumor markers (carbohydrate antigen 19-9 and ... [7] Jun 3, 2022 — Imaging. A variety of imaging tests may be used to diagnose bile duct cancer including those listed below. CT scan: A CT (computed tomography) ... [8] If you do have cholangiocarcinoma, your provider may perform tests on your tissue biopsy and blood (liquid biopsy) to check for genetic changes in cancer cells ... [9] ERCP stands for endoscopic retrograde cholangio pancreatography. It is a test to help diagnose conditions of the liver, bile ducts, pancreas or gallbladder.
Additional Diagnostic Tests
- MRI
- CT scan
- ERCP
- Tissue biopsy
- Bilirubin blood test
- Liver enzymes blood test
- Tumor markers blood test (CA 19-9 and CEA)
Treatment
Treatment Options for Cholangiocarcinoma
Cholangiocarcinoma, also known as bile duct cancer, can be treated with various drug therapies. The goal of these treatments is to slow down or stop the growth of cancer cells.
- Targeted Therapy: Targeted therapy uses drugs or substances that specifically target and attack cancer cells. This approach has shown promise in treating cholangiocarcinoma, especially for patients with advanced disease (1).
- Chemotherapy: Chemotherapy involves using medications to kill cancer cells. The most commonly used chemotherapy agents for cholangiocarcinoma include gemcitabine, cisplatin, capecitabine, oxaliplatin, and 5-fluorouracil (6, 7, 8, 9).
- Pemazyre: Pemazyre (pemigatinib) is a targeted therapy that has been approved by the FDA for treating certain types of previously treated, advanced cholangiocarcinoma (5).
Clinical Trials
New treatments are being tested in clinical trials to improve outcomes for patients with cholangiocarcinoma. For example, a clinical trial is studying the use of tinengotinib, an FGFR inhibitor, which has shown promise in extending survival among patients (2).
Treatment Approaches
The treatment approach for cholangiocarcinoma depends on various factors, including the stage and location of the cancer. Treatment options may include surgery, chemotherapy, targeted therapy, or a combination of these approaches.
- Surgery: Surgery is often recommended when possible to remove as much of the cancer as can be safely removed (4).
- HAI (Hepatic Artery Infusion): HAI involves delivering medication directly into the liver via an implanted pump. This approach has been used in clinical trials and may become a standard treatment for perihilar bile duct cancer in the future (14).
References
- Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells.
- The drug being studied in the clinical trial, tinengotinib, is an example of a next-generation FGFR inhibitor because it binds to the FGFR2 in a unique way.
- This approach does seem to extend survival among patients, especially among those who initially responded to an FGFR inhibitor — the clinical ...
- Treatment. Treatments for cholangiocarcinoma (bile duct cancer) may include: Surgery. When possible, surgeons try to remove as much of the cancer as can be safely removed.
- Pemazyre (pemigatinib) is a targeted therapy that has been approved by the FDA for treating certain types of previously treated, advanced cholangiocarcinoma.
- The most commonly used chemotherapy agents for cholangiocarcinoma include gemcitabine, cisplatin, capecitabine, oxaliplatin, and 5-fluorouracil.
- Chemotherapy involves using medications to kill cancer cells.
- On the basis of PDT and supplemented drug treatment, patients can often avoid the pain of traditional surgery while obtaining good ...
- Most of these drugs can reversibly bind to cysteine residues on the P-loop in the pocket of adenosine triphosphate acid (ATP).
- Selective FGFR inhibitors showed significant clinical activity in patients with advanced cholangiocarcinoma refractory to treatment if patients had acquired the fusion FGFR gene.
- Distal cholangiocarcinoma (bile duct cancer): ... This has been done as part of a clinical trial in the past, and may become a standard treatment for perihilar bile duct cancer in the future.
- Possible risks and side effects: ... The drug used in HAI to treat cholangiocarcinoma is floxuridine.
Recommended Medications
- Chemotherapy
- Targeted Therapy
- Pemazyre (pemigatinib)
- tinengotinib
- gemcitabine
- cisplatin
- Cisplatin
- oxaliplatin
- floxuridine
- Floxuridine
- 5-fluorouracil
- Fluorouracil
- capecitabine
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Cholangiocarcinoma
Cholangiocarcinoma (CCA) is a type of cancer that affects the bile ducts, and its differential diagnosis involves identifying other conditions that may present with similar symptoms. Based on the search results, here are some key points to consider:
- Primary sclerosing cholangitis (PSC): PSC is a chronic inflammatory disease that can lead to bile duct strictures and obstruction, which can mimic CCA [2].
- Hepatocellular carcinoma (HCC): HCC is a type of liver cancer that can present with similar symptoms to CCA, including jaundice and weight loss [5].
- Liver metastases: Liver metastases from other cancers can also be considered in the differential diagnosis of CCA, particularly if there are multiple lesions or a tumor thrombus [5].
- Benign hilar lesions: The differential diagnosis of hilar cholangiocarcinoma (HCCA) and benign hilar lesions remains challenging, but patients with HCCA tend to have an older age at onset [7].
- Metastatic tumors: Metastatic tumors from other parts of the gastrointestinal tract or the rest of the body can also be considered in the differential diagnosis of CCA [8].
Key Features to Consider
When considering the differential diagnosis of CCA, it's essential to note the following key features:
- Age and sex: Patients with HCCA tend to have an older age at onset [7].
- Symptoms: Jaundice, weight loss, and abdominal pain are common symptoms in both CCA and other conditions [5, 6].
- Imaging findings: Cross-sectional imaging can help differentiate between CCA and other conditions, such as HCC or liver metastases [3].
References
[1] May 28, 2024 — Differential Diagnoses · Bile Duct Strictures · Bile Duct Tumors · Biliary Disease · Biliary Obstruction · Cholangitis · Acute Cholecystitis. [2] Jul 1, 2021 — Differential diagnosis for CCA should include primary sclerosing cholangitis (PSC). The chronic inflammation induced by PSC contributes to bile ... [3] by DW Shin · 2023 · Cited by 23 — In patients with intrahepatic lesions, CCA and HCC should be differentiated through cross-sectional imaging and measuring tumor markers. For surgical candidates ... [4] by BE Van Beers · 2008 · Cited by 117 — Cholangiocarcinoma is suspected based on signs of biliary obstruction, abnormal liver function tests, elevated tumor markers (carbohydrate antigen 19-9 and ... [5] Differential diagnosis ; liver metastases. central necrosis (high T2 signal) is more common ; hepatocellular carcinoma (HCC). tumor thrombus more common. capsular ... [6] by RC Lowe · Cited by 11 — DIFFERENTIAL DIAGNOSIS · Jaundice with or without weight loss and abdominal pain · Abdominal pain · Weight loss · Intrahepatic mass ... [7] by L Pang · 2021 · Cited by 4 — The differential diagnosis of HCCA and benign hilar lesions remains difficult. However, we found that patients with HCCA tended to have an older age at onset ... [8] Apr 10, 2022 — The main differential diagnoses include metastatic tumors from other parts of the gastrointestinal tract or the rest of the body as well as ... [9] by DW Shin · 2023 · Cited by 23 — CCA remains a highly fatal malignancy because early diagnosis is difficult. Based on its anatomical location, CCA can be categorized into the following three ... [10] by WJ Lee · 2001 · Cited by 191 — Cholangiocarcinoma is a biliary carcinoma that arises from the intra- and extrahepatic bile ducts and manifests with various histologic types ...
Additional Differential Diagnoses
- Metastatic tumors
- Liver metastases
- Benign hilar lesions
- primary sclerosing cholangitis
- hepatocellular carcinoma
- periampullary adenoma
- autoimmune cholangitis
- ampulla of Vater benign neoplasm
- primary biliary cholangitis
- sclerosing cholangitis
- pancreatic cancer
- opisthorchiasis
- pancreatic ductal adenocarcinoma
- bile duct mucoepidermoid carcinoma
- gallbladder carcinoma
- obsolete adult primary liver cancer
- extrahepatic bile duct leiomyoma
- obsolete unresectable extrahepatic bile duct cancer
Additional Information
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:4947
- core#notation
- DOID:4947
- oboInOwl#hasAlternativeId
- DOID:5249
- oboInOwl#hasDbXref
- UMLS_CUI:C0280725
- IAO_0000115
- A bile duct adenocarcinoma that has_material_basis_in bile duct epithelial cells.
- oboInOwl#hasExactSynonym
- cholangiosarcoma
- rdf-schema#label
- cholangiocarcinoma
- oboInOwl#inSubset
- http://purl.obolibrary.org/obo/doid#NCIthesaurus
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_4928
- relatedICD
- http://example.org/icd10/C22.1
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_11240
- owl#annotatedSource
- t437549
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