Biliary Tract Cancer

 Introduction and Epidemiology

Cholangiocarcinoma is one of the commonest liver cancers worldwide.

Epidemiology 

Three to five cases are reported in the Eldoret Cancer Registry annually with a slight female proponderance. Similar numbers are reported for carcinoma of the gall bladder.

 Diagnosis

Presentation

The commonest presenting symptons are abdominal pain, pruritis and yellowness of eyes. 

Physical examination reveals jaundice and an abdominal mass. 

Investigations include imaging by ultrasound, CT scan and magnetic resonance imaging (MRI).

Others are per cutaneous cholangiography, endoscopic retrograde  cholangiopancreatography (ERCP) and magnetic resonance cholangiopancreatography 
(MRCP) can be used for diagnosis.

Biopsy, fine needle aspiration or biliary brush cytology. A final pathological diagnosis has to be obtained before any chemotherapy, radiotherapy or other non-surgical oncological 
therapy, but is not critical for planning surgery in patients with characteristic findings of  resectable biliary cancer. 

Staging and risk management


Staging consists of complete history and physical examination, blood counts, liver  function tests, chest X-ray, imaging of the abdomen by sonography and CT scan or MRI, 
endoscopic retrograde or percutaneous transhepatic cholangiography and possibly endoscopic ultrasonography, cholangioscopy and laparoscopy. 

Upper and lower GI endoscopy has to be performed in patients with an isolated  intrahepatic mass.

The staging is to be given according to the TNM 2010 system separately  for gallbladder cancer, intrahepatic, perihilar and distal cholangiocarcinoma).

Hilar cholangiocarcinoma (Klatskin’s tumor) is clinically staged depending on the involvement  of the hepatic ducts according to the Bismuth–Corlette classification. 

 TNM Staging of Gallbladder Cancer 
 

Primary Tumor (T)

Tx  Primary tumor cannot be assessed
T0  No evidence of primary tumor
Tis  Carcinoma in situ
T1 Tumor invades lamina propria or muscular layer
T1a  Tumor invades lamina propria
T1b  Tumor invades muscular layer
T2 Tumor invades perimuscular connective tissue; no extension beyond 
 serosa or into liver
T3  Tumor perforates the serosa (visceral peritoneum) and/or directly 
 invades the 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 two or 
 more extrahepatic organs or structures
Regional Lymph nodes
Nx  Regional lymph nodes cannot be assessed
N0  No regional lymph node metastasis
N1 Metastases to nodes along the cystic duct, common bile duct, hepatic 
 artery and/or portal vein
N2  Metastases to periaortic, pericaval, superior mesenteric artery and/or 
 celiac artery lymph nodes
Distant metastasis (M)
M0 No distant metastasis
M1  Distant metastasis

 

 Stage Grouping for Cholangiocarcinoma

Stage 0  Tis, N0, M0
Stage 1  T1, N0, M0
Stage 11  T2,N0,M0
Stage 111a T3, N0,M0
Stage 111b  T1-3, N1,M0
Stage 1Va  T4, N0-1, M0
Stage 1Vb  Any T, N2, M0
Stage 1Vc Any T, Any N, M1

Management

Treatment after incidental finding of gallbladder cancer on pathological review;

Resectable tumors

Complete surgical resection is the only potentially curative treatment available.

Resection  of gallbladder cancer consists of extended cholecystecomy including en bloc hepatic resection and lymphadenectomy (porta hepatis, gastrohepatic ligament, retroduodenal) 
with or without bile duct excision.

Major hepatectomy including caudate lobectomy such  as extended right lobe resection with portal vein resection increases resectability and 
radicality for stage 3 and 4 hilar cholangiocarcinomas and has been associated with higher  5-year survival rates.

Preoperative transarterial or portal vein embolization increases the  remnant liver volume in patients with estimated postresection volumes of <25% and 
appears to reduce postoperative liver dysfunction.

Treatment after incidental finding of gallbladder cancer on pathological review;

Resectable tumors

Complete surgical resection is the only potentially curative treatment available.

Resection of gallbladder cancer consists of extended cholecystecomy including en bloc hepatic  resection and lymphadenectomy (porta hepatis, gastrohepatic ligament, retroduodenal) 
with or without bile duct excision.

Major hepatectomy including caudate lobectomy such  as extended right lobe resection with portal vein resection increases resectability and 
radicality for stage 3 and 4 hilar cholangiocarcinomas and has been associated with higher 5-year survival rates.

Preoperative transarterial or portal vein embolization increases the  remnant liver volume in patients with estimated postresection volumes of <25% and 
appears to reduce postoperative liver dysfunction.

Follow up

Follow-up visits after complete resection should be restricted to history and physical  examination considering symptoms, nutrition and psychosocial problems. 

 Pharmacological management

5-fluorouracil, oxaliplatin, docetaxel, gemcitabine, capecitabine, cisplatin, erlotinib

The combination of 5FU and cisplatin is the preferred first line.

Where resources are  available, gemcitabine and oxaliplatin may be used.

 Prognosis

Even in patients undergoing aggressive surgery, 5-year survival rates are 5–10% for  gallbladder cancer and 10–40% for cholangiocarcinoma.

 References

  1. Biliary cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up; Ann Oncol (2011) 22 (suppl 6): vi40-vi44.
  2. .Aljiffry M, et al. Advances in diagnosis, treatment and palliation of cholangiocarcinoma: 1990-2009. J Gastroenterol 2009;14:4240-4262
  3. Anderson C, Kim R. Adjuvant therapy for resected extrahepatic cholangiocarcinoma: a review of the literature and future directions. Cancer Treat  Rev 2009;35:322-327

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