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Bile Duct Carcinoma
Symposium on Biliary Tract Disease
Bile Duct Carcinoma
James E. Goodnight, [r., M.D., Ph.D. *
Bile duct carcinoma is an uncommon neoplasm, appearing in 0.4 per cent of autopsied patients. The overall prognosis remains poor, but there is a trend toward more aggressive surgical treatment. Klatskin observed that bile duct carcinoma grows slowly and more often causes death from biliary obstruction, liver failure, and cholangitis than from metastatic disease, and that restoration of bile flow extends survival. 8 In this article discussion will be limited to bile duct tumors that occur above the pancreas. Neoplasms in the distal portion of the duct are managed in the same way as cancer of the head of the pancreas but have a somewhat better prognosis.
PATHOLOGY Bile duct carcinoma arises throughout the biliary tree, but more than half the lesions appear in the upper third of the duct, classically at the hepatic hilus. A few patients have multiple lesions. These tumors are invariably adenocarcinomas, although an occasional squamous cell carcinoma is seen. Their growth pattern is local expansion and infiltration, which varies from slow progression that is compatible with lengthy palliation to aggressive proliferation, widespread metastases, and early death. The gross appearance of bile duct carcinomas may be linked to prognosis, with polypoid lesions being more favorable than scirrhous tumors." The latter are more common, and dense fibrosis in the area of the tumor is frequently evident at operation. This scarring can make adequate biopsy difficult to achieve. Bile duct carcinoma appears to be associated with other gastrointestinal diseases. As many as half the patients have a prior history of biliary calculi. A varying number (3 to 11 per cent) have ulcerative colitis, and 3 per cent have a second intra-abdominal primary carcinoma. A weak association exists between carcinoma and cystic disease of the biliary tract.
*Assistant Professor, Department of Surgery, and Chief, Section of Surgical Oncology, University of California, Davis, School of Medicine, Sacramento, California
Surgical Clinics of Nortli America-Vol. 61, No.4, August 1981
981
982
JAMES
E.
GOODNIGHT, JR.
PRESENTATION Growth of this tumor is most often silent until it obstructs the flow of bile. Nearly all patients present with obstructive jaundice. Half have disagreeable epigastric pain and significant weight loss. The mean age of presentation is between 60 and 65, but every series has patients in their 30s. The onset of jaundice is usually abrupt, but some patients become severely debilitated from insidious growth of the tumor and chronic hyperbilirubinemia before their tumor is recognized. Cholangitis is a feature of the disease but is an unusual presenting complaint. Hemobilia occurs but is rare. At operation for obstructive jaundice, the surgeon must be alert for a collapsed, stone-free gallbladder and distal common duct.
DIAGNOSIS Bile duct carcinoma is diagnosed in the work-up for obstructive jaundice. Percutaneous transhepatic cholangiography (PTC) is the most helpful study, since it outlines the upper biliary duct and usually the tumor itself. In addition, a catheter can be left in place for decompression of the biliary tree. With current techniques, bleeding, bile leakage, and cholangitis are infrequent. 7 • 13 Bile duct cancers can also be visualized with angiography, ultrasonography, and endoscopic retrograde cholangiography, but these studies usually do not add significantly to the information gained from PTC. Computerized axial tomographic (CAT) scans will outline extraductal extensions of the tumor, liver metastases, and subhepatic masses and can improve operative planning. If the scan raises the question of metastatic disease, barium studies of the upper gastrointestinal tract and the colon and sigmoidoscopy are indicated. Establishing the diagnosis preoperatively is helpful. Patients appear to do better if treated definitively at the initial operation. At operation, the gross findings are usually obvious, but the fibrous reaction can make histologic confirmation difficult. Biopsy through the choledochoscope or gentle scrapings with a scoop are the preferred diagnostic maneuvers. If PTC has not been done in the preoperative work-up, intraoperative transhepatic cholangiography should be performed to outline the ducts and tumor.
TREATMENT Resection of the Primary Tumor When it can be accomplished, the most desirable treatment of bile duct carcinoma is complete excision of the lesion. Unfortunately, this approach is possible in less than half the cases, and even when it is accomplished, most patients ultimately die of the disease. Nevertheless, the patients who enjoy the longest survival and greatest freedom from hospitalization are those in whom the tumor has been completely resected." 5. 9 Moreover, the curative potential of alternative treatments is essentially nonexistent, and prolongation of survival from palliative procedures is usually limited. As experience with
BILE DUCT CARCINOMA
983
liver surgery has grown, the number of successful resections of bile duct cancer including a portion or all of one lobe of the liver has increased. Evander and colleagues resected the primary tumor in 27 of 80 patients (34 per cent) and Launois and his associates reported complete removal of 11 of 18 carcinomas at the hepatic hilus." 9 Their operative mortality rates were 11 and 20 per cent, respectively, which is significant but no greater than those of other operations for the disease. In general, if no metastases are present and the longevity of the patient is not limited by some other disease, an aggressive attempt to resect the tumor is warranted. Obviously, the success of this approach depends on the skill and experience of the individual surgeon. The most frequent complication is leakage or stricture of the biliaryenteric anastomosis (26 per cent in Evander's series). Severely debilitated patients are not candidates for radical resection. Preoperative decompression of the biliary tree via external drainage reduces the risk of sepsis, liver failure, and uncontrollable hemorrhage.
Hepatic-Enteric Bypass Procedures Despite the desirability of resecting this tumor, most cannot be completely removed because of extension to the portal vessels or metastases to the liver parenchyma. Nevertheless, growth of this tumor is frequently slow and localized so that effective palliation and prolongation of survival can be provided by relieving obstruction to bile flow. This effort circumvents hepatic failure and cholangitis. Selection and performance of the appropriate palliative procedure requires the best surgical judgment and skill. The number of options available indicates the lack of satisfaction with any single procedure. The advent of routine PTC adds an option to the palliative armamentarium. Biliary drainage through an external catheter placed at the time of this procedure can effectively lower the level of serum bilirubin and relieve symptoms. Once the bile ducts are cannulated in this fashion, successively larger tubes can be passed to provide good egress of bile. Moreover, it is frequently possible to pass the indwelling tube through the neoplasm into the distal common duct and duodenum to provide internal drainage of bile. The patient irrigates the tube, usually every other day, to reduce encrustation. Once a tract is formed, the tubes can be changed with relative ease. Bouts of cholangitis occur in most patients who have long-term indwelling catheters, but the episodes are controlled with antibiotics, Hemobilia is an occasional problem. Drainage of one side of the liver usually suffices, but patients with severe compromise of liver function may need to have both sides drained. Debilitated patients who are poor operative risks can benefit from percutaneous tube drainage and a few have survived more than a year with their catheter in place. Unfortunately for most, the benefit is short-lived (median is 2 to 4 months),": 13 In the past, a commonly employed procedure to restore bile flow was passage of one limb of a T-tube or similar drainage tube through the tumor. Because bile encrustation obstructed these tubes, periodic changes via laparotomy were required. Terblanche has described a U-tube arrangement that appears to be superior." At laparotomy, the distal common duct is opened. A number 3 Bakes dilator is passed proximally to locate the tumor, which is then examined with a choledochoscope, and biopsy is performed. The stric-
984
JAMES
E.
GOODNIGlIT, JR.
tured area is carefully dilated, then the instrument is passed into a proximal dilated duct, most often on the right side. The dilator is forced through the surface of the liver, a heavy silk thread tied to it, and the dilator is withdrawn. One end of an appropriate tube (Terblanche uses the Argyle Leveen tube) is attached to the thread and pulled through the liver and stricture and. out the common duct. Holes cut in the tube are positioned to lie above and below the obstruction, thus providing internal drainage. Both ends of the tube are passed through separate stab wounds in the abdominal wall and secured by tying each to a crosspiece of tubing. Initially, both ends are drained, but later they are connected to each other. The common duct is closed around the tube and the area is drained. The patient irrigates the tube daily and becomes an expert in its management. If the tube is obstructed or dislodged, it can be changed by tying a new tube to the first and railroading the second tube through. The new tube is positioned by radiopaque markers on its surface. This convenience is the major advantage of the V-tube. Terblanche reports long-term survival following this procedure, particularly when it is combined with radiation therapy. Internal decompression of the biliary tree with an hepatic-enteric anastomosis potentially has significant advantages over tube arrangements. Such procedures may avoid the complications of external tube drainage, which are discomfort, the need for management of the tube, hemobilia, cholangitis, and superficial infections. Longmire and Sanford described resection of the lateral portion of the left lobe of the liver to expose a dilated bile duct, removal of a core of surrounding liver tissue, and anastomosis of the duct to a Roux-enY loop of jejunun.!' Cahow has recently described a somewhat simpler cholangiojejunostomy." A subcapsular bile duct is identified with a needle, which is a relatively easy procedure to perform in this situation. This duct is exposed, cannulated, and followed to a larger, but still relatively superficial, duct 1 to 2 em within the liver. The larger duct is incised longitudinally and a small saucer-shaped portion of the liver is removed to facilitate anastomosis. A Roux-en-Y limb of jejunun is brought up and anastomosed side-to-side to the bile duct. To obtain a more precise anastomosis, a portion of the jejunal serosa is removed and the submucosa and mucosa are sewn to the bile duct. The serosa is sutured to the liver capsule. In both techniques, the anastomosis is stented by a tube that is placed across the anastomosis and brought through the Roux-en-Y limb and out the abdominal wall. In his initial 14 patients, Cahow had 3 postoperative deaths (21 per cent)." These deaths occurred in elderly, debilitated patients. His contraindications to the procedure now are marked debilitation of the patient, hepatic metastases, portal hypertension, and absence of dilatation of the ducts. The average survival following the procedure is 16 months. This compares favorably with mean survivals of 20 and 24 months reported by Evander et al. and Lanunois et al. for resection of these tumors.v 9 For appropriately selected patients, internal drainage offers excellent palliation and freedom from external tube management.
Liver Transplantation Because bile duct carcinoma involves the liver almost exclusively, removal of the liver and orthotoptic liver transplantation has been performed for selected patients. Calne reported 12 cases of liver transplantation for this disease, but all 12 patients developed recurrent tumor is less than one year
985
BILE DUCT CARCINOMA
and succumbed." Starzl has one patient surviving disease-free longer than a year." At this time, liver transplantation is an experimental procedure, and benefit to patients with bile duct carcinoma has been very limited.
Radiation Therapy The role of radiation therapy for bile duct carcinoma is unclear. Investigators reporting on palliative treatment for this disease indicate that their longest survivors have had radiation to the tumor as an adjunct to relief of biliary tract obstruction. Terblanche reports two survivors of greater than six years following placement ofa U-tube and radiation to the tumor.F Lees and colleagues indicated an increase in median survival of patients undergoing radiation treatment of bile duct carcinorna.!" However, these results are purely anecdotal and some long-term survivors have had drainage procedures alone. Nevertheless, in the absence of data to the contrary, it is reasonable to consider radiating the primary tumor in patients who are provided effective bypass of their biliary obstruction. Whenever possible, the tumor should be outlined by clips at laparotomy to help define the radiation port.
Chemotherapy As with most carcinomas of the gastrointestinal tract, the only anticancer drug with an established record of activity is 5-fluorouracil, and its activity is limited. Responses to the drug have been recorded, but the extent of data is small. There is no information to indicate that 5-fluorouracil or other drugs given as an adjunct to surgery or radiation are beneficial. However, an attempt to treat measurable metastatic disease with 5-fluorouracil is reasonable. If the patient's tumor responds, symptoms may improve and survival may increase. If there is not an obvious response, drug treatment should be discontinued. Patients with hepatic metastases from this disease generally have very limited survival.
PROGNOSIS Overall, the results of treatment for this disease are unsatisfactory and prognosis is poor. Nevertheless, it is clear that quality and length of survival can be improved by relieving obstruction to bile flow. Revealing information regarding prognosis in 80 cases of bile duct carcinoma was presented by Evander et al. 5 Patients with radical resection for cure (12 of 80, the group with the most favorable outlook), spent a median 26 per cent of remaining life in the hospital. Median survival was 20 months. Those patients whose tumor was incompletely removed but who received a functional hepatic-enteric bypass (15 of 80) spent a median of 46 per cent of remaining life in a hospital. Median survival was 7.5 months. The patients whose disease was not resected (53 of 80) spent a median of 97 per cent of remaining life in the hospital. Median survival was 2.5 months. Obviously, within each group there was considerable variation, and there was a 36 month survivor among the patients whose tumor was not resected. Patients have lived three to five years, or longer, following either resection of the tumor or palliative bypass, which indicates that some patients' lives are extended and the quality is improved. Therefore, an aggressive approach to this disease is warranted.
986
JAMES
E.
GOODNIGHT, JR.
SUMMARY Bile duct carcinoma most often causes death by obstructing bile flow; relief of this obstruction extends survival. Options include passage of tubes through the tumor to maintain internal bile drainage, hepatic-enteric bypass, and percutaneous tube drainage. As experience with liver surgery has grown, the number of bile duct carcinomas that have been successfully resected has increased. Complete removal of the tumor produces more long-term survivors than other procedures, but most patients still succumb to their disease. Nevertheless, aggressive treatment to excise the lesion or bypass the biliary obstruction significantly improves quality and length of life for many patients.
REFERENCES 1. Andersson, A., Bergdahl, L., and Van der Linden, W.: Malignant tumors of the extrahepatic bile ducts. Surgery, 81: 198-202, 1977. 2. Akwari, O. E., and Kelly, K. A.: Surgical treatment of adenocarcinoma of bile duct. Arch. Surg., 114:22-25, 1979. 3. Calne, R. Y.: Transplantation of the liver. Ann. Surg., 188:129-138,1978. 4. Cahow, C. E.: Intrahepatic cholangiojejunostomy: A new simplified approach. Am. J. Surg., 137:443, 1979. 5. Evander, A., Fredlund, P., Hoevels, J., et al.: Evaluation of aggressive surgery for carcinoma of the extrahepatic bile ducts. Ann. Surg., 191 :23-29, 1980. 6. Fortner, J. G., Kallum, B. 0., and Kim, D. K.: Surgical management of carcinoma of the junction of the main hepatic ducts. Ann. Surg., 184 :68-73, 1976. 7. Hansson, J. A., Hoevels, J., Simert, G., et al.: Clinical aspects of nonsurgical percutaneous transheptic bile drainage in obstructive lesions of the extrahepatic bile ducts. Ann. Surg., 189:58-61, 1979. 8. Klatskin, G.: Adenocarcinoma of the hepatic duct bifurcation. Am. J. Med., 38:241, 1965. 9. Launois, B., Campion, J.-P., Brissot, P., et al.: Carcinoma of the hepatic hilus. Ann. Surg., 190:151-157, 1979. 10. Lees, C. D., Zapolanski, A., Cooperman, A. M., et al.: Carcinoma of the bile ducts. Surge Gynecol. Obstet., 151: 193-198, 1980. 11. Longmire, W. P., and Sanford, M. C.: Intrahepatic cholangiojejunostomy with partial hepatectomy for biliary obstruction. Surgery, 24 :264, 1948. 12. Longmire, W. P., McArthur, M. S., Bastounis, E. A., et al.: Carcinoma of the extrahepatic biliary tract. Ann. Surg., 178 :333-345, 1973. 13. Pollock, T. W., Ring, E. R., Oleaga, J. A., et al.: Percutaneous decompression of benign and malignant biliary obstruction. Arch. Surg., 114:148-151, 1979. 14. Ross, A. P., Braasch, J. 'W., and Warren, K. W.: Carcinoma of the proximal bile ducts. Surge Gynecol. Obstet., 136:923, 1973. 15. Starzl, T. E.: Hepatic transplantation. Surgery, 79:727, 1976. 16. Takasan, H., Kim, C. I., Arii, S., et al.: Clinicopathologic study of 70 patients with carcinoma of biliary tract. Surge Gynecol. Obstet., 150:721-726, 1980. 17. Terblanche, J.: Carcinoma of the proximal extrahepatic biliary tree. Surge Annu., 11 :249-265, 1979. 18. Todoroki, T., Okamura, T., Fukao, K., et al.: Gross appearance of carcinoma of the main hepatic duct and its prognosis. Surge Gynecol. Obstet., 150:33-40, 1980. Section of Surgical Oncology School of Medicine University of California, Davis 4301 X Street Sacramento, California 95817
Bile Duct Carcinoma
James E. Goodnight, [r., M.D., Ph.D. *
Bile duct carcinoma is an uncommon neoplasm, appearing in 0.4 per cent of autopsied patients. The overall prognosis remains poor, but there is a trend toward more aggressive surgical treatment. Klatskin observed that bile duct carcinoma grows slowly and more often causes death from biliary obstruction, liver failure, and cholangitis than from metastatic disease, and that restoration of bile flow extends survival. 8 In this article discussion will be limited to bile duct tumors that occur above the pancreas. Neoplasms in the distal portion of the duct are managed in the same way as cancer of the head of the pancreas but have a somewhat better prognosis.
PATHOLOGY Bile duct carcinoma arises throughout the biliary tree, but more than half the lesions appear in the upper third of the duct, classically at the hepatic hilus. A few patients have multiple lesions. These tumors are invariably adenocarcinomas, although an occasional squamous cell carcinoma is seen. Their growth pattern is local expansion and infiltration, which varies from slow progression that is compatible with lengthy palliation to aggressive proliferation, widespread metastases, and early death. The gross appearance of bile duct carcinomas may be linked to prognosis, with polypoid lesions being more favorable than scirrhous tumors." The latter are more common, and dense fibrosis in the area of the tumor is frequently evident at operation. This scarring can make adequate biopsy difficult to achieve. Bile duct carcinoma appears to be associated with other gastrointestinal diseases. As many as half the patients have a prior history of biliary calculi. A varying number (3 to 11 per cent) have ulcerative colitis, and 3 per cent have a second intra-abdominal primary carcinoma. A weak association exists between carcinoma and cystic disease of the biliary tract.
*Assistant Professor, Department of Surgery, and Chief, Section of Surgical Oncology, University of California, Davis, School of Medicine, Sacramento, California
Surgical Clinics of Nortli America-Vol. 61, No.4, August 1981
981
982
JAMES
E.
GOODNIGHT, JR.
PRESENTATION Growth of this tumor is most often silent until it obstructs the flow of bile. Nearly all patients present with obstructive jaundice. Half have disagreeable epigastric pain and significant weight loss. The mean age of presentation is between 60 and 65, but every series has patients in their 30s. The onset of jaundice is usually abrupt, but some patients become severely debilitated from insidious growth of the tumor and chronic hyperbilirubinemia before their tumor is recognized. Cholangitis is a feature of the disease but is an unusual presenting complaint. Hemobilia occurs but is rare. At operation for obstructive jaundice, the surgeon must be alert for a collapsed, stone-free gallbladder and distal common duct.
DIAGNOSIS Bile duct carcinoma is diagnosed in the work-up for obstructive jaundice. Percutaneous transhepatic cholangiography (PTC) is the most helpful study, since it outlines the upper biliary duct and usually the tumor itself. In addition, a catheter can be left in place for decompression of the biliary tree. With current techniques, bleeding, bile leakage, and cholangitis are infrequent. 7 • 13 Bile duct cancers can also be visualized with angiography, ultrasonography, and endoscopic retrograde cholangiography, but these studies usually do not add significantly to the information gained from PTC. Computerized axial tomographic (CAT) scans will outline extraductal extensions of the tumor, liver metastases, and subhepatic masses and can improve operative planning. If the scan raises the question of metastatic disease, barium studies of the upper gastrointestinal tract and the colon and sigmoidoscopy are indicated. Establishing the diagnosis preoperatively is helpful. Patients appear to do better if treated definitively at the initial operation. At operation, the gross findings are usually obvious, but the fibrous reaction can make histologic confirmation difficult. Biopsy through the choledochoscope or gentle scrapings with a scoop are the preferred diagnostic maneuvers. If PTC has not been done in the preoperative work-up, intraoperative transhepatic cholangiography should be performed to outline the ducts and tumor.
TREATMENT Resection of the Primary Tumor When it can be accomplished, the most desirable treatment of bile duct carcinoma is complete excision of the lesion. Unfortunately, this approach is possible in less than half the cases, and even when it is accomplished, most patients ultimately die of the disease. Nevertheless, the patients who enjoy the longest survival and greatest freedom from hospitalization are those in whom the tumor has been completely resected." 5. 9 Moreover, the curative potential of alternative treatments is essentially nonexistent, and prolongation of survival from palliative procedures is usually limited. As experience with
BILE DUCT CARCINOMA
983
liver surgery has grown, the number of successful resections of bile duct cancer including a portion or all of one lobe of the liver has increased. Evander and colleagues resected the primary tumor in 27 of 80 patients (34 per cent) and Launois and his associates reported complete removal of 11 of 18 carcinomas at the hepatic hilus." 9 Their operative mortality rates were 11 and 20 per cent, respectively, which is significant but no greater than those of other operations for the disease. In general, if no metastases are present and the longevity of the patient is not limited by some other disease, an aggressive attempt to resect the tumor is warranted. Obviously, the success of this approach depends on the skill and experience of the individual surgeon. The most frequent complication is leakage or stricture of the biliaryenteric anastomosis (26 per cent in Evander's series). Severely debilitated patients are not candidates for radical resection. Preoperative decompression of the biliary tree via external drainage reduces the risk of sepsis, liver failure, and uncontrollable hemorrhage.
Hepatic-Enteric Bypass Procedures Despite the desirability of resecting this tumor, most cannot be completely removed because of extension to the portal vessels or metastases to the liver parenchyma. Nevertheless, growth of this tumor is frequently slow and localized so that effective palliation and prolongation of survival can be provided by relieving obstruction to bile flow. This effort circumvents hepatic failure and cholangitis. Selection and performance of the appropriate palliative procedure requires the best surgical judgment and skill. The number of options available indicates the lack of satisfaction with any single procedure. The advent of routine PTC adds an option to the palliative armamentarium. Biliary drainage through an external catheter placed at the time of this procedure can effectively lower the level of serum bilirubin and relieve symptoms. Once the bile ducts are cannulated in this fashion, successively larger tubes can be passed to provide good egress of bile. Moreover, it is frequently possible to pass the indwelling tube through the neoplasm into the distal common duct and duodenum to provide internal drainage of bile. The patient irrigates the tube, usually every other day, to reduce encrustation. Once a tract is formed, the tubes can be changed with relative ease. Bouts of cholangitis occur in most patients who have long-term indwelling catheters, but the episodes are controlled with antibiotics, Hemobilia is an occasional problem. Drainage of one side of the liver usually suffices, but patients with severe compromise of liver function may need to have both sides drained. Debilitated patients who are poor operative risks can benefit from percutaneous tube drainage and a few have survived more than a year with their catheter in place. Unfortunately for most, the benefit is short-lived (median is 2 to 4 months),": 13 In the past, a commonly employed procedure to restore bile flow was passage of one limb of a T-tube or similar drainage tube through the tumor. Because bile encrustation obstructed these tubes, periodic changes via laparotomy were required. Terblanche has described a U-tube arrangement that appears to be superior." At laparotomy, the distal common duct is opened. A number 3 Bakes dilator is passed proximally to locate the tumor, which is then examined with a choledochoscope, and biopsy is performed. The stric-
984
JAMES
E.
GOODNIGlIT, JR.
tured area is carefully dilated, then the instrument is passed into a proximal dilated duct, most often on the right side. The dilator is forced through the surface of the liver, a heavy silk thread tied to it, and the dilator is withdrawn. One end of an appropriate tube (Terblanche uses the Argyle Leveen tube) is attached to the thread and pulled through the liver and stricture and. out the common duct. Holes cut in the tube are positioned to lie above and below the obstruction, thus providing internal drainage. Both ends of the tube are passed through separate stab wounds in the abdominal wall and secured by tying each to a crosspiece of tubing. Initially, both ends are drained, but later they are connected to each other. The common duct is closed around the tube and the area is drained. The patient irrigates the tube daily and becomes an expert in its management. If the tube is obstructed or dislodged, it can be changed by tying a new tube to the first and railroading the second tube through. The new tube is positioned by radiopaque markers on its surface. This convenience is the major advantage of the V-tube. Terblanche reports long-term survival following this procedure, particularly when it is combined with radiation therapy. Internal decompression of the biliary tree with an hepatic-enteric anastomosis potentially has significant advantages over tube arrangements. Such procedures may avoid the complications of external tube drainage, which are discomfort, the need for management of the tube, hemobilia, cholangitis, and superficial infections. Longmire and Sanford described resection of the lateral portion of the left lobe of the liver to expose a dilated bile duct, removal of a core of surrounding liver tissue, and anastomosis of the duct to a Roux-enY loop of jejunun.!' Cahow has recently described a somewhat simpler cholangiojejunostomy." A subcapsular bile duct is identified with a needle, which is a relatively easy procedure to perform in this situation. This duct is exposed, cannulated, and followed to a larger, but still relatively superficial, duct 1 to 2 em within the liver. The larger duct is incised longitudinally and a small saucer-shaped portion of the liver is removed to facilitate anastomosis. A Roux-en-Y limb of jejunun is brought up and anastomosed side-to-side to the bile duct. To obtain a more precise anastomosis, a portion of the jejunal serosa is removed and the submucosa and mucosa are sewn to the bile duct. The serosa is sutured to the liver capsule. In both techniques, the anastomosis is stented by a tube that is placed across the anastomosis and brought through the Roux-en-Y limb and out the abdominal wall. In his initial 14 patients, Cahow had 3 postoperative deaths (21 per cent)." These deaths occurred in elderly, debilitated patients. His contraindications to the procedure now are marked debilitation of the patient, hepatic metastases, portal hypertension, and absence of dilatation of the ducts. The average survival following the procedure is 16 months. This compares favorably with mean survivals of 20 and 24 months reported by Evander et al. and Lanunois et al. for resection of these tumors.v 9 For appropriately selected patients, internal drainage offers excellent palliation and freedom from external tube management.
Liver Transplantation Because bile duct carcinoma involves the liver almost exclusively, removal of the liver and orthotoptic liver transplantation has been performed for selected patients. Calne reported 12 cases of liver transplantation for this disease, but all 12 patients developed recurrent tumor is less than one year
985
BILE DUCT CARCINOMA
and succumbed." Starzl has one patient surviving disease-free longer than a year." At this time, liver transplantation is an experimental procedure, and benefit to patients with bile duct carcinoma has been very limited.
Radiation Therapy The role of radiation therapy for bile duct carcinoma is unclear. Investigators reporting on palliative treatment for this disease indicate that their longest survivors have had radiation to the tumor as an adjunct to relief of biliary tract obstruction. Terblanche reports two survivors of greater than six years following placement ofa U-tube and radiation to the tumor.F Lees and colleagues indicated an increase in median survival of patients undergoing radiation treatment of bile duct carcinorna.!" However, these results are purely anecdotal and some long-term survivors have had drainage procedures alone. Nevertheless, in the absence of data to the contrary, it is reasonable to consider radiating the primary tumor in patients who are provided effective bypass of their biliary obstruction. Whenever possible, the tumor should be outlined by clips at laparotomy to help define the radiation port.
Chemotherapy As with most carcinomas of the gastrointestinal tract, the only anticancer drug with an established record of activity is 5-fluorouracil, and its activity is limited. Responses to the drug have been recorded, but the extent of data is small. There is no information to indicate that 5-fluorouracil or other drugs given as an adjunct to surgery or radiation are beneficial. However, an attempt to treat measurable metastatic disease with 5-fluorouracil is reasonable. If the patient's tumor responds, symptoms may improve and survival may increase. If there is not an obvious response, drug treatment should be discontinued. Patients with hepatic metastases from this disease generally have very limited survival.
PROGNOSIS Overall, the results of treatment for this disease are unsatisfactory and prognosis is poor. Nevertheless, it is clear that quality and length of survival can be improved by relieving obstruction to bile flow. Revealing information regarding prognosis in 80 cases of bile duct carcinoma was presented by Evander et al. 5 Patients with radical resection for cure (12 of 80, the group with the most favorable outlook), spent a median 26 per cent of remaining life in the hospital. Median survival was 20 months. Those patients whose tumor was incompletely removed but who received a functional hepatic-enteric bypass (15 of 80) spent a median of 46 per cent of remaining life in a hospital. Median survival was 7.5 months. The patients whose disease was not resected (53 of 80) spent a median of 97 per cent of remaining life in the hospital. Median survival was 2.5 months. Obviously, within each group there was considerable variation, and there was a 36 month survivor among the patients whose tumor was not resected. Patients have lived three to five years, or longer, following either resection of the tumor or palliative bypass, which indicates that some patients' lives are extended and the quality is improved. Therefore, an aggressive approach to this disease is warranted.
986
JAMES
E.
GOODNIGHT, JR.
SUMMARY Bile duct carcinoma most often causes death by obstructing bile flow; relief of this obstruction extends survival. Options include passage of tubes through the tumor to maintain internal bile drainage, hepatic-enteric bypass, and percutaneous tube drainage. As experience with liver surgery has grown, the number of bile duct carcinomas that have been successfully resected has increased. Complete removal of the tumor produces more long-term survivors than other procedures, but most patients still succumb to their disease. Nevertheless, aggressive treatment to excise the lesion or bypass the biliary obstruction significantly improves quality and length of life for many patients.
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