Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening. 

Current RMA Instruments:

Changes from previous instruments:    

ICD coding:

• Malignant neoplasm of the intra-hepatic bile duct (ICD-10 C22.1)
• Malignant neoplasm of the extra-hepatic bile duct (ICD-10 C24.0)
• Malignant neoplasm of the ampula of Vater (ICD-10 C24.1)

Brief description:

Malignant neoplasm of the bile duct, also known as cholangiocarcinoma, is a rare and aggressive form of cancer that arises from the epithelial cells of the intrahepatic or extrahepatic bile ducts (including the ampulla of Vater and the cystic duct). These ducts are responsible for carrying bile from the liver to the gallbladder and small intestine. 

Cholangiocarcinoma can occur anywhere along the bile ducts and is classified according to its location. Perihilar cholangiocarcinoma (Klatskin tumor) is the most common type, accounting for 50-60% of all cases. Overall, in terms of histology, the majority of cholangiocarcinomas are adenocarcinomas (subtype papillary, nodular or diffuse/sclerosing) with the rest being squamous cell carcinomas.

Confirming the diagnosis:

To confirm the diagnosis there needs to histology of the bile duct tumour. High resolution imaging may be suggestive but not definitive. Note that it is important to establish that the tumour is malignant, it is a primary tumor of the bile duct and that it is not a sarcoma, lymphoma or carcinoid.

It does not need to be symptomatic.

The relevant medical specialist is a gastroenterologist, oncologist or general surgeon.

Additional diagnoses covered by these SOPs

• Cholangiocarcinoma 
• Klatskin tumour (hilar cholangiocarcinoma)
• Adenosquamous carcinoma of the bile duct
• Squamous cell carcinoma of the bile duct
• Undifferentiated carcinoma of the bile duct
• Lymphoepithelioma-like cholangiocarcinoma
• Adenocarcinoma of ampulla of Vater
• Malignant neoplasm of the ampulla of Vater
• Malignant neoplasm of the common bile duct
• Malignant neoplasm of the common hepatic duct
• Malignant neoplasm of the cystic duct
• Malignant neoplasm of the hepatic duct

Conditions not covered by these SOPs

• Benign neoplasm of the bile duct #
• Carcinoid tumour #
• Neuroendocrine carcinoma of the gallbladder #
• Hodgkin lymphoma *
• Malignant neoplasm of the gallbladder *
• Non-Hodgkin lymphoma *
• Pancreatic duct cancer (Malignant neoplasm of the pancreas) *
• Secondary malignant neoplasm (use SOP for primary site)
• Soft tissue sarcoma*

* another SOP applies

^# non-SOP condition

Clinical onset

The assessment of the clinical date of onset begins with the positive histopathology, then goes back in time to the date of positive imaging, and if possible goes back to the onset of clinical symptoms and signs which are consistent with the neoplastic process. This type of malignancy commonly results in reports of painless jaundice (yellowing of the skin and eyes), right upper quadrant abdominal pain and weight loss. Painless jaundice is considered the most specific sign associated with cholangiocarcinoma. 

Individuals may sometimes have this cancer but be asymptomatic, with this malignancy being discovered incidentally during the process of investigating other conditions. Despite the lack of symptoms, the date of onset could be taken from this time. 

Clinical worsening

It is difficult to ascertain whether a malignant neoplasm has clinically worsened out of keeping with the natural history of the underlying pathology. This condition is also usually picked up when the malignancy has reached an advanced stage. Seeking the advice of the treating oncologist would be recommended for any assessment of possible clinical worsening.