Hass (not real name, M847) is a 44-year-old male from Indonesia.  In early 2010, he had jaundice. The doctor suspected this was due to Hepatitis. Hass was prescribed medication. After a month, the treatment was not effective. Hass went to a private hospital in Kuching.

A CT scan on 28 January 2010 indicated a 6 mm stone in the gallbladder. There was a 1 cm hypodense lesion in segment 8 of his liver. This could be just a cyst.  The common bile duct was dilated. The maximum diameter was 16 mm. There was a 18 mm mass at the distal common duct. Conclusion: Mass in the lower common duct with biliary obstruction.

Immediately Hass underwent an operation to remove his gallbladder and the bile duct tumour. The histopathology report dated 3 February 2010 indicated: infiltrating moderately differentiated exophytic adenocarcinoma of bile duct, invading through entire thickness of the wall and metastatic to periductal lymph nodes. Margins of excision free of tumour.

An ultrasound of the abdomen on 8 June 2010 showed no evidence of tumour recurrence although his CA19.9 increased from 52.4 (in March 2010) to 1420.0. A CT scan on 9 June 2010 indicated a 15 x 10mm lesion in segment 7 of his liver. The 10 mm cyst in segment 8 of his liver remained the same. In addition there were a few paraaortic lymph nodes noted from the celiac axis down to the liver of the renal hilum. The largest node measured 20.0 mm – the others were subcentimeter.

The doctor told Hass that the cancer had recurred and he was unable to help him anymore. Hass was asked to undergo chemotherapy. But he refused.

Below is a table showing the rise of CA 19.9 markers from March to October 2010.

Hass came to see us in Penang on 8 October 2010. Listen to our conversation.

Review of Medical Literature

Understanding the Liver-Gallbladder-Pancreas-Bile Duct Complex

• Attached to the liver, is a pear-shaped structure called the gallbladder.
• The liver makes bile and this is transported to the intestine by a long tube-like structure called the bile duct.
• The gallbladder is attached to the bile duct by a small tube called the cystic duct.
• The top half of the bile duct is associated with the liver while the bottom half of the bile duct is associated the pancreas through which it passes on its way to the intestine.
• Blockage of the bile duct by a cancer prevents the bile from being transported to the intestine. When this happens the bile accumulates in the blood giving rise to jaundice. The skin and eyes becomes yellow. This condition may also cause severe itchiness.

(Diagrams are obtained from the internet)

The clinical presentation, prognosis and natural history of bile duct cancer (or cholangiocarcinoma) depend on where the cancer originates. The cancer may occur:

• In the part of the bile ducts contained within the liver. This is known as intra-hepatic.
• In the part of the bile ducts outside the liver. This is known as extra-hepatic.
• In the duct between the left and right portal veins. This is known as hilar bifurcation.

Anderson et al. in Diagnosis and treatment of cholangiocarcinoma, The Oncologist, Vol. 9, No. 1, 43–57, February 2004, noted that approximately:

• 60%-70% of cholangiocarcinomas occur at the hilar bifurcation.
• 20%-30% in the distal common bile duct.
• 5%-15% within the liver (intra-hepatic).

Patients with extrahepatic tumors usually present with painless jaundice due to biliary obstruction, while patients with intrahepatic tumors usually present with pains, pruritus (66%), abdominal pain (30%-50%), weight loss (30%-50%), and fever (up to 20%).

In their review, Anderson et al also noted that:

• The majority of patients with cholangiocarcinoma presents with unresectable,advanced stage disease and have a survival of less than 12 months. Cure rates are low, even with aggressive therapy. The average 5-year survival rate is 5%-10%.
• Surgery remains the only intervention offering the possibility of a cure. But the treatment goal should be complete excision with negative margins.
• All patients should be fully evaluated for resectability before any type of intervention is performed.
• Neoadjuvant and adjuvant therapies have not improved survival in patients with this tumor.
• Since the prognosis for unresectable patients is poor, palliative measures should be aimed at increasing quality of life first and increasing survival second.

Results of Surgery

• Among selected patients who undergo potentially curative resections, 5-year survival rates are generally from 8%-44%.
• The importance of achieving a margin-negative resection cannot be overemphasized. The 5-year survival rates were greater when a negative margin was obtained, 19%-47% versus 0%-12%.
• Distal lesions represent approximately 20%-30% of all cholangiocarcinomas and are usually treated with pancreaticoduodenectomy (Whipple procedure). Achieving a margin-negative resection is also important with these tumors. The 5-year survival rates in selected patients who underwent curative resections is 21%-54%.
• Intrahepatic cholangiocarcinoma is usually treated by hepatic resection. Three-year survival rate of 60% was reported in patients who underwent a margin-negative partial hepatectomy.

Chemotherapy

• Chemotherapy has not been shown to markedly improve survival in patients with either resected or unresected cholangiocarcinoma. The majority of reports use 5-FU alone or in combination with methotrexate, leucovorin, cisplatin, mitomycin C, or interferon alpha.
• The 5-year survival rates were not significantly different between patients who received chemotherapy and surgery and those who received surgery alone.

A note in http://emedicine.medscape.com/article/189843-treatment stated  that:  “Chemotherapy has not been proven to be of definite benefit.”

Experience of doctors in Fuda Hospital, China indicated that:

• Cancer arising in the extrahepatic bile duct is an uncommon disease, curable by surgery in fewer than 10% of all cases.
• In most patients, the tumor cannot be completely removed by surgery and is incurable.
• Palliative resections or other palliative measures e.g. stenting procedures may maintain adequate biliary drainage and allow for improved survival.

Experience of doctors in India indicated that:

• Surgery may be used to remove the cancer if it has not spread beyond the bile duct. It is not always possible to carry out surgery, as the bile duct is in a difficult position and it may be impossible to remove the cancer completely.
• There are different surgical procedures depending upon how big the cancer is and whether it has begun to spread into nearby tissues.

These surgical procedures are:

• Removal of the bile ducts. If the cancer is small and contained within the ducts, then just the bile ducts containing the cancer are removed.
• Partial liver resection. If the cancer has begun to spread into the liver, the affected part of the liver is removed, along with the bile ducts.
• Whipple’s. If the cancer is larger and has spread into nearby structures, then the bile ducts, part of the stomach, part of the duodenum, the pancreas, gall bladder and the surrounding lymph nodes are all removed.
• Bypass surgery. Sometimes it is not possible to remove the tumour. Other procedures may be performed to relieve the blockage and allow the bile to go into the intestine. The jaundice will then clear up.
• Stent insertion. One way to relieve jaundice without a surgical operation is to perform ERCP (Endoscopic Retrograde Cholangiopancreatography). This procedure involves insertion of a tube, called a stent into the blocked bile duct. The stent clears a passage through the bile duct to allow the bile to drain away. The tube usually needs to be replaced every three to four months to prevent it becoming blocked. If the tube does block, recurrent high temperatures and/or return of the jaundice will occur.

In summary, Witzigmann et al. in an article, Guidelines for palliative surgery of cholangiocarcinoma, HPB (Oxford). 2008 June 1; 10(3): 154–160, listed the following guidelines for surgery of bile duct cancer:

1. Non-surgical stenting is regarded as the first choice of palliation for patients with short life expectancy.
2. For patients with longer projected survival, surgical bypass should be considered.
3. Palliative resections may be beneficial to patients with distal and hilar bifurcation.

Questions to Ponder

The fact is very clear. Surgery for bile duct cancer in this case is an absolute failure. Is this failure a rare event? Probably NOT! A surgeon told his liver patient: “For ten liver operations that I performed, nine patients died”. Good information indeed but the problem is that he made this comment some three years too late. This patient had already undergone the operation and had suffered a severe recurrence.

A few more questions come to mind:

• According to the literature, not all patients with bile duct cancer are suitable condidates for surgery.  “All patients should be fully evaluated for resectability before any type of intervention is performed”. To what extent is this evaluation done?
• The surgeon recommended chemotherapy after the recurrence. What benefit can the patient expect from this? Medical literature clearly indicates that “Chemotherapy has not been proven to be of definite benefit.”
• Is the suggestion of chemotherapy after the failure of surgery, a way of “passing the buck” to someone else? “I have done my job to cut it out. My responsibility is over. Now you go to the oncologist and see what he can do for you.”

More related stories:

Liver Cancer: Surgery and Chemo Did Not Cure Him

A Great Failure and Let Down

Surgery for Liver Cancer: Eighty Percent Success?

Liver Cancer: He Died After a RM 10,000 Open-Close Surgery

Healing of Metastatic Liver Cancer