Malignant biliary disease--diagnosis and management

Malignant biliary disease-diagnosis and management

1. Malignant Biliary Diseases

2. Cholangiocarcinoma Three distinct pathologic subtypes: sclerosing , nodular, and papillary Sclerosing cholangiocarcinoma Proximal bile ducts, causing periductal fibrosis in a concentric pattern and a circumferential duct occlusion Firm mass based in the duct wall can be seen growing into lumen Papillary and nodular Distal cholangiocarcinomas and are manifested with intraluminal growths. I Papillary - polypoid lesion that is soft, less periductal fibrosis, better prognosis Three staging subdivisions include intrahepatic, extrahepatic , and distal bile duct Direct local invasion and local lymph node spread indicate worse prognosis

3. Risk Factors Chronic inflammation and compensatory cellular proliferation Choledochal cysts E xposure of the biliary epithelium to pancreatic secretions More prevalent in Southeast Asia L iver flukes  Clonorchis sinensis  and  Opisthorchis viverrini creates Recurrent pyogenic cholangitis (primary bile duct stone formation) PSC A utoimmune multifocal strictures of the intrahepatic and extrahepatic biliary trees More likely to have multifocal disease not amenable to resection Medications and chemical carcinogens Thorotrast , oral contraceptives, asbestos, and cigarette smoke

4. Presentation Depends on the site of origin Painless jaundice is a common symptom Unilobar obstruction of a bile duct Lobar atrophy and subsequent contralateral lobar hypertrophy Hepatic compensation can delay presentation Obstruction at or below the hepatic bifurcation tends to manifested at earlier stages Direct hyperbilirubinemia Pruritus Dark urine Steatorrhea

5. Left Lobar Atrophy with Ductal Dilation

6. Diagnosis Labs Hyperbilirubinemia and elevated alkaline phosphatase Prothrombin time and albumin level, are generally unaffected until later CEA and CA 19-9 unreliable for diagnosis Followed postoperatively for recurrence

7. Diagnosis R UQ ultrasound Intrahepatic biliary ductal dilation Hilar cholangiocarcinomas GB and extrahepatic biliary tree decompressed Distal lesions Extrahepatic biliary ductal dilation and GB distention Triphasic CT Assessment of metastatic disease Evaluation of resectability Location of the tumor Relationship to vascular structures Identification of aberrant anatomy Segmental or lobar involvement for preoperative planning Cholangiography (MRCP, PTC, or ERCP)-determine the proximal extent

8. Resectability Unresectable diease Bilobar intrahepatic metastases Extrahepatic disease Encasement of the main portal vein Bilateral hepatic lobar artery involvement Lobar atrophy with involvement of the contralateral portal vein or biliary radicals

9. Treatment Operative candidates should have exploration 7 %-15 % will have benign disease >50 % will be unresectable Distal cholangiocarcinoma . Pancreaticoduodenectomy with frozen section of the proximal bile duct margin 5 -year survival rates of up to 50% in node-negative patients with an R0 resection Proximal cholangiocarcinoma Resection of regional nodal tissue and en bloc resection of CBD with hepatic parenchyma to negative margins

10. Bismuth- Corlette classification

11. Type I and II Common duct resection, cholecystectomy, 5-10 -mm margin of resection Type II lesions may need partial hepatic resection Skeletonization of the hepatic artery and portal vein Reconstruction with Roux limb of jejunum

12. Type III and IV Resection and reconstruction Portal vein, hepatic artery, or both Transanastomotic stenting for healing and patency Negative margin status is the most important variable associated with outcome. Five-year survival rates as high as 59% with R0

13. Medical Treatment Chemotherapy and radiation have not been shown to improve survival Some retrospective studies-small survival advantage with adjuvant radiation No prospective studies have shown no benefit

14. Palliation Relieve of jaundice Endoscopic(distal) of percutatneous drainage (proximal) Alleviate of pain Narcotics or celiac plexus block Relieve of duodenal obstruction Endoscopic stenting Surgical palliation has not been shown to prolong survival or to reduce complication rates Reserved for candidates found to be unresectable or metastatic at time of operation

15. Gallbladder Cancer No specific presenting symptoms, presentation with late-stage disease is common 90% of originate in the fundus or body Weight loss, jaundice, abdominal mass in later stages Corresponds with poor prognosis Earlier stage disease warrant aggressive surgical approach Overall survival of gallbladder cancer is less than 15 months

16. Incidence Sixth and seventh decades of life More common in women than in men Highest incidence in women from India and Pakistan North American populations: Native Americans and immigrants from Latin America

17. Causes C hronic inflammation with subsequent cellular proliferation Presence of gallstones is considered to be the primary risk factor Larger stones (>3 cm) carry an increased risk of cancer development > 80% of patients with gallbladder cancer have cholelithiasis Gallbladder cancer 7x more common in patients with gallstones Other risk factors Choledochal cysts P orcelain gallbladder G allbladder polyp larger than 10 mm Less than 10% of patients with porcelain gallbladder will develop cancer

18. Pathology and Staging Mostly adenocarcinoma Progression Dysplasia to carcinoma in situ to invasive carcinoma Papillary subtype carry a significantly better prognosis Commonly limited to the gallbladder wall at the time of diagnosis Nodal disease in 35% and distant metastases in 40%. Nodal basin- hepatoduodenal ligament M ay include periaortic nodes near the celiac axis or pancreaticoduodenal nodes around the superior mesenteric artery

19. Pathology and Staging CT scan P eritoneal metastases H epatic parenchymal metastases Lymphadenopathy A djacent vascular involvement-hepatic arterial or portal venous Triphasic -Cholangiography-location of obstruction Unresectability P ortal vein encasement E xtensive hepatic involvement H epatic or peritoneal metastases

20. Treatment Resection of gallbladder cancer only potential for cure  Neither chemotherapy nor radiation therapy has shown a survival benefit

21. Treatment: Incidental findings Gallbladder polyp - polyps larger than 10 mm O pen cholecystectomy Gallbladder cancer after cholecystectomy T1a lesions- carcinoma penetrates the lamina propria but does not invade the muscle layer Cholecystectomy T1b - penetrating the muscularis but not the deeper connective tissue or serosa Cholecystectomy sufficient if margins negative

22. Treatment T1b treatment: Perineural , lymphatic, or vascular invasion Extended cholecystectomy with removal of pericholedochal , periportal , hepatoduodenal , right celiac, and posterior pancreaticoduodenal lymph nodes Resect cystic duct margin to uninvolved mucosa May require resection of the CBD with Roux-en- Y 2 cm of apparently normal hepatic parenchyma from fossa All port sites should also be excised

23. T2 lesions-extends past the muscularis but not beyond the serosa Radical cholecystectomy as 40% have lymph node metastases Treatment: Incidental findings

24. Treatment Preoperative work-up to evaluate for resectibility Diagnostic laparoscopy to identify peritoneal or hepatic metastases Radical resection for T3 and T4 lesions At least segments IVB and V May require central hepatectomy (IV, V, and VIII) or right trisegmentectomy Direct extension requires en bloc resection with negative margins Debulking has no role 

25. Treatment Patients with advanced disease at presentation Goal is palliation of symptoms Jaundice -endoscopic biliary stenting Pain -narcotics, percutaneous neurolysis of the celiac ganglion Intestinal obstruction-endoscopic duodenal wall stent

26. Survival T1a lesions and T1b lesions to negative margins have excellent prognosis T2 lesions depends on nodal status Radical resection improves 5-year survival from ~20% to more than 60% T3 leisons - 5-year survival less than 20 %