Cholangiocarcinoma

 

Median survival: 6-12 months

 

 

                                     Epidemiology:

  • – It generally occurs in patients >65 years
  • – Average age at diagnosis 50 years
  • – Peak incidence in eighth decade
  • – Uncommon before the age 40 years except in case of PSC

                                   Pathologic findings:

  • Extrahepatic cholangiocarcinoma- 80-90%
  •               Perihilar/hilar/klatskin tumor
  •               Distal cholangiocarcinoma
  • Intrahepatic cholangiocarcinoma (peripheral cholangiocarcinoma) – 10%
  • Macroscopic subtypes of extrahepatic cholangiocarcinoma
  •                 Sclerosing (mass forming)
  •                 • Nodular (Periductal infiltrating)
  •                 Papillary (Intraductal)
  • Hilar cholangiocarcinomas are mostly periductal infiltrating and spreads along the Glisson’s sheath via lymphatic
  • Intrahepatic cholangiocarcinomas are mostly mass forming and tends to invade hepatic parenchyma
  •                            Figure: Macroscopic Subtypes of Extrahepatic Cholangiocarcinoma

 

 

  •                           Figure: Bismuth-Corlette classification of Klatskin tumor

 

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  •            Histological types of Cholangiocarcinoma:
  •                      – Adenocarcinoma- 90%
  •                      – Intestinal-type
  •                      – Clear-cell adenocarcinoma
  •                      – Signet-ring cell carcinoma
  •                      – Adenosquamous carcinoma
  •                      – Squamous cell carcinoma
  •                      – Small cell carcinoma

 

                                                      CLINICAL FEATURES:

• Hilar Cholangiocarcinoma

  • –  Painless progressive jaundice; Papillary lesion may cause fluctuating jaundice
  • –  Bacterial cholangitis
  •  Hepatomegaly, palpable GB, ascites

• Intrahepatic Cholangiocarcinoma

  • – Abdominal pain
  • – Cachexia, malaise, fatigue

                 

                               

                                                          INVESTIGATIONS

  • – Tumor marker: CA:19-9 (>129 U/ml, 98% specific)
  • – USG, CT scan, MRCP
  •                              
  •                                 EUS
  • • Detects tumor dimension and anatomic location in relation to surroundung structures
  • Regional lymphadenmopathy
  • EUS guided FNAC from the lymph node
  • EUS guided biopsy of the primary lesion is highly discouraged due to potential tumor cell spread
  • – ERCP with biliary brush cytology
  • FISH of cytology may demonstrate
  •              • Trisomy 7
  •               Tetrasomy or duplication of all labelled chromosome
  •               Polysomy or amplification of at least 3 chromosomes

**Polysomy is diagnostic of cholangiocarcinoma in the appropriate setting

** Trisomy 7 is not diagnostic but increase the patient’s risk for cholangiocarcinoma

  • NEWER TECHNIQUES FOR DIAGNOSING CHOLANGIOCARCINOMA:
  •         – Choledochoscopy (cholangioscopy) and cholangioscopy directed biopsy
  •         – Intrductal US
  •         – Choledochoenteroscopy
  •                                 

Figure: Diagnostic algorithm for perihilar cholangiocarcinom

 

                    Staging of cholangiocarcinoma:

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  •                          TREATMENT OF CHOLANGIOCARCINOMA

 

                                                                 Surgery

  •                                   
  •                                    Surgical resection for perihilar cholangiocarcinoma
  • – Lobar/ extended lobar hepatic and biliary duct resection + Regional lymphadenectomy + Roux-en-y hepaticojejunostomy
  • – Patients with PSC and perihilar cholangiocarcinoma should be considered for liver transplantation instead of resection

 

 

  •                                                    
  •                  
  •                                                      Surgical resection for distal cholangiocarcinoma:
  •                                                                            -Whipple resection
  • ** Adjuvant/ neoadjuvant chemo/radio therapy is not recommended
    • Prognosis after R0, N0 resection
    •         – 5 year survival for hilar cholangiocarcinoma: 11 – 67%
    •         – 5 year survival for hilar cholangiocarcinoma: 27-37%
    •        – R0 resectability rates are <50%

 

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  •               Palliative therapy
  • – For biliary drainage: Endoscopi, percutaneous, surgical
  • – Photodynamic therapy: Reduces cholestasis, improves quality of life
  • – External beam radiation therapy
  • – Intraoperative/ intraductal brachytherapy

 


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