Cholangiocarcinoma ICD 10 Code

Refresher: What are ICD-10 Codes?

ICD-10 stands for the International Classification of Diseases, 10th revision. It is a coding system used in healthcare to systematically classify diagnoses, procedures, and symptoms. CM indicates “Clinical Modification” and is used in the US for disease diagnoses (ICD-10-CM). ICD-10-PCS codes are used for coding procedures in inpatient settings. Both of these coding systems are used for medical claims and statistics purposes.

The International Classification of Diseases was developed in the 1850s as a system to standardize medical records and data using a single coding system. The ICD-10 (10th edition) began in 1983, with the World Health Organization managing the official list of codes.

Countries that have adopted the ICD-10 have modified them based on their specific healthcare system requirements. Currently, there are over 70,000 ICD-10-PCS codes and around 68,000 ICD-10-CM codes used for defining malignant neoplasms. [1]

The US version of the ICD-10 was constructed in collaboration with the National Center for Health Statistics (NCHS) and the Centers for Medicare & Medicaid Services (CMS).

What is the ICD 10 Code for Cholangiocarcinoma?

The ICD-10 code for cholangiocarcinoma is C22.1 [2]. All tissue in the body is made up of cells. Cancer is a medical condition in which the cells in the body begin to multiply at an alarming rate. This, in turn, results in the development of tumors in the area where the cells begin to multiply uncontrollably. These cancerous cells not only destroy other healthy cells but can also spread to nearby organs via the lymph system. Bile duct carcinoma is a rare condition in which cancerous cells grow in the bile ducts. Cholangiocarcinoma is another name for bile duct cancer.

The small intestine, gallbladder, and liver are linked by a network of tubes known as ducts. The network originates in the liver, where several tiny ducts collect bile (a fluid produced by the liver to aid in the breakdown of lipids during digestion). The tiny ducts join to create the right and left hepatic ducts, which exit the liver. Outside the liver, the two channels connect to create the common hepatic duct, and the gallbladder is connected to it through the cystic duct. Bile from the liver is transported to the gallbladder via the hepatic ducts, common hepatic duct, and cystic duct.

Primary Cholangiocarcinoma ICD 10 Code: C22.1

Cholangiocarcinoma is categorized based on where it is located in relation to the liver. An Intrahepatic cholangiocarcinoma develops in the liver's tiny bile ducts. This is the disease's least frequent form, accounting for fewer than 10% of all cases. The hilum, where the right and left main bile ducts to unite and leave the liver, is where perihilar cholangiocarcinoma (also known as a Klatskin tumor) originates. It accounts for more than half of all cases and is the most frequent type of the disease.

The remaining instances are distal cholangiocarcinomas, which start in bile ducts outside the liver. The disease's perihilar and distal variants, which both develop outside the liver, are frequently clubbed together and referred to as extrahepatic cholangiocarcinoma.

Cholangiocarcinoma is a kind of cancer that develops in the thin tubes (bile ducts) that transport the digestive fluid bile. The bile ducts connect the liver to the gallbladder and the small intestine. Cholangiocarcinoma, often known as bile duct cancer, primarily affects persons over the age of 50, but it can develop at any age. Doctors categorize cholangiocarcinoma into three categories based on where cancer develops in the bile ducts:

Intrahepatic cholangiocarcinoma is a kind of liver cancer that develops in portions of the bile ducts within the liver.

Hilar cholangiocarcinoma is a kind of cancer that develops in the bile ducts right outside the liver. This is also known as perihilar cholangiocarcinoma.

Distal cholangiocarcinoma develops in the bile duct towards the liver.

The liver is an organ that produces bile, which is then transported to the gallbladder through the bile ducts. The bile is stored in the gallbladder, and when we ingest something, the bile is then released into the intestines.

/0 – The tumor is benign.

/1 – It is not known for sure whether the tumor is benign or malignant.

/2 – Early form of cancer

/3 – It is malignant cancer.

/6 – It is a metastasis.

/9 – It is either malignant cancer or metastasis. The cells in the affected tissue are altered and multiply uncontrollably.

Primary Cholangiocarcinoma ICD Code C22.1 Lookup

Cholangiocarcinoma, also known as bile duct cancer, is a rare and aggressive type of cancer. The biliary system, also known as the bile duct system, is a series of tubes that originate from the liver and end in the small intestine.

Basically, bile is a fluid that's used in the digestive system to break down foods. Abdominal pain, unintentional weight loss, and jaundice are some of the common symptoms of cholangiocarcinoma. While the exact causes of cholangiocarcinoma are yet unknown, factors such as age or a rare condition such as primary sclerosing cholangitis may increase one's chances of getting bile duct cancer.

C22.1: Malignant Neoplasm of Cholangiocarcinoma

Types of Cholangiocarcinoma Coded Under C22.1

C22.1 is a billable ICD-10 CM code that is used for diagnosis for all reimbursements. While the US version of the ICD-10 code 22.1 is used for billing purposes in the US, the ICD-10 code may change in international versions. The two types of cholangiocarcinoma include malignant neoplasm of the extrahepatic bile duct and intrahepatic bile duct cancer. Extrahepatic bile duct cancer can be further divided into two types; distal bile duct and perihilar bile duct cancer.

Cancer develops when a series of mutations in essential genes, such as those that govern cell division, cause cells to proliferate and divide uncontrolled, resulting in the formation of a tumor. These genetic alterations are found only in the bile duct cells that give birth to the tumor in the majority of instances of cholangiocarcinoma.

Somatic mutations are genetic alterations that are not inherited. Cholangiocarcinoma has been reported to have somatic mutations in a variety of genes. Some of these genes function as tumor suppressors, which means they assist in maintaining cell growth and division under strict control. Mutations or deletions of tumor suppressor genes can cause cells to proliferate and divide uncontrollably, which is a characteristic of cancer.

Cholangiocarcinoma Excluded from ICD-10 C22.1

The ICD-10 code 22.1 excludes the medical conditions; malignant neoplasm of biliary tract NOS (C24.9) and secondary malignant neoplasm of the liver and intrahepatic bile duct (C78.7).

Secondary and Metastatic Cholangiocarcinoma ICD 10 Code: C22.1

Cholangiocarcinoma is a rare tumor that originates in the biliary tree. The tumors are difficult to diagnose and treat and, more often than not, have a poor prognosis. Over the past few years, studies have identified molecular heterogeneity with a success rate of up to 50% for detectable tumors.[3]

Secondary or Metastatic Cholangiocarcinoma ICD-10 Code C22.1 Lookup

As the disease progresses, advanced intrahepatic cholangiocarcinomas often metastasize to other organs in the body, mainly the liver, brain, bones, as well as lungs. Anatomically, cholangiocarcinoma is divided into two sub-types which are mainly; extrahepatic cholangiocarcinoma and intrahepatic cholangiocarcinoma, based on the cancer's location. For cholangiocarcinoma that has been successfully diagnosed early on, the 5-year survival rate is around 24%. However, if the cholangiocarcinoma has spread to the lymph nodes, then the 5-year survival rate drops down to 9%.

Cholangiocarcinoma does not run in families. According to research, the blood relations of a patient may have a higher chance of having the disease than the general population. However, most cholangiocarcinoma patients do not have a family history of the disease.

Experts are unsure what causes cholangiocarcinoma. The risk factors indicate that persistent inflammation in the bile ducts may have a role in the development of this malignancy. Persistent damage, such as inflammation, can also cause DNA alterations, which can alter how particular cells grow, divide, and behave. These modifications are most likely not inherited, which implies that parents do not pass them on to their offspring. Changes are more likely to occur over a person's lifespan.

Conclusion

Cholangiocarcinoma is a cancer that develops in the biliary epithelium. However, these tumors may occur at any location along the biliary tree. It is important to note that cancer of the bile duct may be considered part of the cholangiocarcinoma family but requires a different clinical and therapeutic approach.