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Malignant Neoplasm of the Bile Duct B010

Document
Last amended 
30 June 2015

Current RMA Instruments:

Reasonable Hypothesis SOP
69 of 2015
Balance of Probabilities SOP
70 of 2015
Changes from previous instruments:    

SOP Bulletin 183

ICD coding:
  • Intra-hepatic bile duct (ICD-9 155.1; ICD-10 C22.1)
  • Extra-hepatic bile duct (ICD-9 156.1; ICD-10 C24)
  • Ampula of Vater (ICD-9 156.2; ICD-10 C24.1)
Brief description:

This is a malignant neoplasm of the biliary tree which drains bile from the liver into the small intestine with temporary storage in the gall bladder. The biliary tree starts inside the liver as the right and left hepatic ducts which drain into the common hepatic duct which exits the liver and joins with the cystic duct (connected to the gallbladder) then becoming the common bile duct which finally joins to the 2nd part of the duodenum with the terminal part of the common bile duct called the ampulla of Vater.  The terminal part of the common bile duct joins with the pancreatic duct arising from the pancreas.

Histology of the bile duct malignant neoplasm (cholangiocarcinoma) is of variable type being mainly (90%) adenocarcinoma (subtype papillary, nodular or diffuse/sclerosing) and 10% 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 neoplastic, that the neoplasm is malignant, that the malignant neoplasm is a primary 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 of the bile duct
  • 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#
  • Hodgkin’s lymphoma*
  • Malignant neoplasm of the gallbladder*
  • Non-Hodgkin’s 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 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. The usual clinical presentation is painless jaundice, right upper quadrant abdominal pain and weight loss, with the jaundice being the most specific sign.

Note that bile duct neoplasia is usually silent, so the neoplasm may have been discovered incidentally during some other investigation procedure. As such the clinical onset is the date of this incidental investigation, unless other overlooked pathognomonic symptoms or signs can take the clinical onset further back in time.

If the client suffers with biliary obstruction, then the clinical presentation of the biliary colic and obstructive jaundice will likely be the true clinical onset.

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. Further given the very nature of the neoplasm, the diagnosis is usually made when the condition is advanced.