Ch. 54 – Biliary System

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1580 Section X Abdomen Box 54-7 Risk Factors for Gallbladder Carcinoma Gallstones Porcelain gallbladder Anomalous pancreatobiliary junction Choledochal cysts Adenomatous gallbladder polyps Primary sclerosing cholangitis Obesity Salmonella typhi infection in Chile, the incidence of gallbladder cancer is particularly high. Etiology Several factors have been associated with an increased risk for developing gallbladder cancer (Box 54-7). Although not entirely clear, the pathogenesis is likely related to chronic infl ammation. Among these factors, gallstones are the most common because of the high prevalence in the general population. The association between an APBDJ, a porcelain gallbladder, and other biliary disorders such as choledochal cysts and primary sclerosing cholangitis and gallbladder cancer has been recognized more recently. A strong association has long been noted between gallbladder cancer and cholelithiasis, which is present in 75% to 90% of cases. The incidence of gallstones increases with age, and by age 75, about 35% of women and 20% of men in the United States have developed gallstones. The incidence of gallbladder cancer is about seven times more common in the presence of cholelithiasis and chronic cholecystitis than in people without gallstones. In addition, the risk for developing gallbladder cancer is higher in patients with symptomatic gallstones than in patients with asymptomatic gallstones. About 1% of all elective cholecystectomies performed for cholelithiasis harbor an occult gallbladder cancer. Pathology and Staging Ninety percent of cancers of the gallbladder are classifi ed as adenocarcinoma. Squamous cell, oat cell, undifferentiated, and adenosquamous cancers and carcinoid tumors are much less frequent. Six percent of gallbladder adenocarcinomas demonstrate papillary features histopathologically; these tumors are commonly diagnosed while localized to the gallbladder and are also associated with an improved overall survival. At diagnosis, 25% of cancers are localized to the gallbladder wall, 35% have associated metastases to regional lymph nodes or extension into adjacent organs, and 40% have already metastasized to distant sites. Lymphatic drainage from the gallbladder occurs in a predictable fashion and correlates with the pattern of lymph node metastases seen in gallbladder cancer. Hepatic involvement with gallbladder cancer can occur by direct invasion through the gallbladder bed, angiolymphatic portal tract invasion, or distant hematogenous Table 54-2 TNM Staging for Gallbladder Cancer T1 Tumor invades lamina propria (T1a) or muscular (T1b) layer T2 Tumor invades perimuscular connective tissue, no extension beyond the serosa or into the liver T3 Tumor perforates the serosa (visceral peritoneum) and/or directly invades into liver and/or one other adjacent organ or structure such as the stomach, duodenum, colon, pancreas, omentum, or extrahepatic bile ducts T4 Tumor invades main portal vein or hepatic artery or invades multiple extrahepatic organs and/or structures N0 No lymph node metastases N1 Regional lymph node metastases M0 No distant metastases M1 Distant metastases Stage Stage Grouping IA T1 N0 M0 IB T2 N0 M0 IIA T3 N0 M0 IIB T1 N1 M0 T2 N1 M0 T3 N1 M0 III T4 Any N M0 IV Any T Any N M1 Adapted from Greene F, Page D, Fleming I, et al (eds): AJCC Cancer Staging Manual, 6th ed. New York, Springer-Verlag, 2002. spread. The current TNM classifi cation of the American Joint Committee on Cancer (AJCC) is shown in Table 54-2. The appropriate management and overall prognosis are strongly dependent on tumor stage. Clinical Presentation Gallbladder cancer most often presents with right upper quadrant abdominal pain often mimicking cholecystitis and cholelithiasis. Weight loss, jaundice, and an abdominal mass are less common presenting symptoms. About 40% of patients present with symptoms of chronic cholecystitis, often with a recent change in the quality or frequency of the painful episodes. Another common presentation is similar to acute cholecystitis, with a short duration of pain associated with vomiting, fever, and tenderness. Signs and symptoms of malignant biliary obstruction with jaundice, weight loss, and right upper quadrant pain are also common. Patients can also present with symptoms of a nonbiliary malignancy with anorexia and weight loss in the absence of jaundice or, least commonly, with signs of gastrointestinal bleeding or obstruction. Gallbladder cancer is often misdiagnosed as chronic cholecystitis, pancreatic cancer, acute cholecystitis, choledocholithiasis, or gallbladder hydrops. Diagnosis Ultrasonography is often the fi rst diagnostic modality used in the evaluation of patients with right upper quad-
ant abdominal pain. A heterogeneous mass replacing the gallbladder lumen and an irregular gallbladder wall are common sonographic features of gallbladder cancer. The sensitivity of ultrasound in the detection of gallbladder cancer ranges from 70% to 100%. CT scan usually demonstrates a mass replacing the gallbladder or extending into adjacent organs. Spiral CT also demonstrates the adjacent vascular anatomy. With newer magnetic resonance techniques, gallbladder cancers may be differentiated from the adjacent liver and biliary obstruction or encasement of the portal vein may also be easily visualized. Cholangiography also may be helpful in diagnosing jaundiced patients with gallbladder cancer. The typical cholangiographic fi nding in gallbladder cancer is a long stricture of the common hepatic duct. Angiography, spiral CT, or MRI may identify encasement of the portal vein or hepatic artery. If radiographic studies suggest that the tumor is unresectable (liver or peritoneal metastases, portal vein encasement, or extensive hepatic invasion), a biopsy of the tumor is warranted and can be performed under ultrasound or CT guidance. Management The appropriate operative procedure for the patient with localized gallbladder cancer is determined by the pathologic stage. Patients with tumors confi ned to the gallbladder mucosa or submucosa (T1a) or confi ned to the gallbladder muscularis (T1b) are usually identifi ed after cholecystectomy for gallstone disease and have an overall 5-year survival rate approaching 100% and 85%, respectively. Therefore, cholecystectomy is adequate therapy for patients with T1 tumors. Recurrent cancer at port sites and peritoneal carcinomatosis have been reported after laparoscopic cholecystectomy, even for patients with in situ disease; therefore, all port sites should be excised if a patient has had a previous laparoscopic cholecystectomy. Bile spillage occurs in 26% to 36% of laparoscopic cholecystectomies and appears to be even more common (50%) in cases of gallbladder cancer. Spillage is associated with poor survival even in early stage (T1 and T2) gallbladder cancer. Patients with preoperatively suspected gallbladder cancer should undergo open cholecystectomy to minimize the chance of bile spillage and tumor dissemination. Cancer of the gallbladder with invasion beyond (stages II and III) the gallbladder muscularis is associated with an increased incidence of regional lymph node metastases and should be managed with an “extended cholecystectomy.” This includes lymphadenectomy of the cystic duct, pericholedochal, portal, right celiac, and posterior pancreatoduodenal lymph nodes. Obtaining an R0 resection should be the goal of surgery and results in an improved survival compared with patients who have remaining microscopic or macroscopic disease. 42 Adequate clearance of the pericholedochal lymph nodes may be facilitated by resection of the common bile duct, but common duct resection is not always necessary; in those cases in which the cystic duct stump margin is positive for malignancy, common duct resection with Roux-en-Y reconstruction is mandatory. Extension into the hepatic Chapter 54 Biliary System 1581 parenchyma is common, and extended cholecystectomy should incorporate at least a 2-cm margin beyond the palpable or sonographic extent of the tumor. For smaller tumors, this goal can be achieved with a wedge resection of the liver. For larger tumors, an anatomic liver resection (extended right hepatectomy) may be required to achieve a histologically negative margin. Staging laparoscopy should be performed in patients with gallbladder cancer, as a high proportion (50%-55%) of patients have hepatic or extrahepatic disease that is not detected by noninvasive staging modalities. In most cases, therapy for gallbladder cancer is palliative. If a tissue diagnosis can be established in patients with an unresectable tumor, nonoperative palliation should be considered. Many of these patients have obstructive jaundice that can be managed with either an endoscopic or percutaneously placed biliary stent. Pain is another problem that should be treated aggressively to improve quality of life. Percutaneous celiac ganglion nerve block may reduce the need for narcotics. The results of chemotherapy in the treatment of patients with gallbladder cancer have been quite poor. External-beam and intraoperative radiation therapy have both been used in the management of patients with gallbladder cancer. Unfortunately, no randomized data have demonstrated improved survival with either chemotherapy or radiation. Survival Survival in patients with gallbladder cancer is strongly infl uenced by the pathologic stage at presentation. Recent advances in surgical technique and aggressiveness of resection for gallbladder cancer have resulted in improved overall survival 42 (Fig. 54-33). Patients with cancer limited to the gallbladder mucosa and lamina propria (T1a) have an excellent prognosis. Invasion into the muscular wall (T1b) of the gallbladder increases the risk for recurrent cancer after curative resection. However, no difference % Alive 100 75 50 25 0 P < .03 0 20 40 60 Survival (months) 80 TP 2 TP 1 100 Figure 54-33 Improved survival due to an aggressive approach to gallbladder cancer comparing two time periods (TPs), 1990- 1996 and 1996-2002 (circles) (P < .03). (From Dixon E, Vollmer CM, Sahajpal A, et al: An aggressive surgical approach leads to improved survival in patients with gallbladder cancer: A 12-year study at a North American Center. Ann Surg 241:385-394, 2005.)
Page 1 and 2: Biliary System Ravi S. Chari, MD an
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Ch. 54 – Biliary System

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