Cholangiocarcinoma: Pathology, diagnosis and treatment.

Cholangiocarcinoma

Marco Castillo

Definition. Uncommon cancer.

Anywhere along the biliary system.◦ 40 – 60% in the hilum (Klatskin tumors).◦ 20 – 30% in the distal biliary lower tract.◦ 10% Intrahepatic.◦ 10% Multifocal.

Bad prognosis.

Bismuth-Corlette classification of hilar cholangiocarcinoma.

Type I Type II

Type IIIa Type IIIb

Type IV

Epidemiology 1 – 2 of 100.000 in the US.

More common in:◦ Native American. Japanese.

> 65 years. Men > Women.

Risk factors:◦ Primary Sclerosing Cholangitis.◦ Choledochal cyst.◦ Prior transduodenal sphincteroplasty.◦ Biliary parasitic infection.

◦ Clornochis sinensis. ◦ Opisthorchis viverrini

◦ Teratogens: Asbestos, Nitrosamines, Thorotrast, Dioxin.

Pathology.

Poorly differentiated Adenocarcinoma.Desmoplastic reaction.Longitudinal growth.

Classification:◦ Mass forming.◦ Periductal-infiltrating,◦ Intraductal-papillary.

Pathology.

Pathology.CLINICAL PRESENTATION.

Painless Jaundice.

Direct hyperbilirubinemia.

Pain.

Dark urine.

Acholia.

Cholangitis.

Steatorrhea.

Anorexia.

Weight loss.

DIAGNOSIS.

Contrast CT or MRI.Shows arterial and Venous enhancement.

Cholangiography. MRCP. ERCP.

CA-19

AJCC Staging System for Cholangiocarcinoma

AJCC Staging System for Cholangiocarcinoma

AJCC Staging System for Cholangiocarcinoma

Treatment: UNRESSECTABILITY CRITERIA:

Medical contraindication to surgical intervention

Advanced cirrhosis/portal hypertension.

Bilateral second-order biliary involvement.

Main portal vein involvement.

Lobar atrophy with contralateral second-order biliary radicle involvement.

Lobar atrophy with contralateral portal vein involvement.

N2 nodal involvement.

Distant metastases.

Treatment:SURGICAL TREATMENT:

Intrahepatic cholangiocarcinoma Hepatic segmentectomy o lobectomy.

Distal extrahepatic Pancreatoduedenectomy.

Hiliar Biliar duct resection +/- Hepatic lobectomy.

PSC Neoadjuvant + Radiation + Liver transplantation.

Goal is to decrease pain and jaundice symptoms.

ERCP o Percutaneous stent.

PALLIATION:

Clinical pearls: Cholangiocarcinoma is a disease with different clinical presentation.

CT with contrast and MRI are the best diagnostic tools for Cholangiocarcinoma, followed by Cholangiography.

Classification and localization of the Cholangiocarcinoma determines treatment and prognosis.

Surgical resection, is the best treatment available.

Patients without resectability criteria should be evaluated for Neoadjuvant therapy and Palliation.

Bibliography Cho S, Fong Y. Biliary Tract Tumors. In: Shackelford’s Surgery of the Alimentary Tract. 7th edition. Pp: 1364 – 1379.

Jackson PG and Evans S. Biliary System. In: Sabiston Textbook of Surgery. 20th Edition. Pp: 1482 – 1519.

Blechacz B and Gores GJ. Tumors of the Bile Ducts, Gallbladder and Ampulla. In: Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 5th Edition.Pp: 1184-1200.

Nakanuma Y, Sato Y, Harada K. Pathological classification of intrahepatic cholangiocarcinoma based on a new concept. World J Hepatol. 2010 Dec 27; 2(12): 419–427.