Cholangiocarcinoma

Question 1

general prognosis

Answer 1

highly lethal (most are advanced at presentation)

Question 2

Symptomatology and physiology of extrahepatic cholangiocarcinomas

Answer 2

• become symptomatic when tumor obstructs biliary drainage system
• jaundice, pruritus, clay-colored stools, dark urine, abdominal pain, weight loss

Question 3

Difference in lab presentation between extrahepatic and intrahepatic cholangiocarcinoma

Answer 3

Extrahepatic = biliary obstruction patten (high t bili, direct bili, and alk phos), normal transaminases initially and then can be elevated due to chronic obstruction

Intrahepatic = high ALP, serum bili normal or slightly elevated

Question 4

Imaging of extrahepatic biliary cancer + imaging feature suggesting cholangio

Answer 4

Often small and can be missed, but ductal dilatation in absence of stone is suggestive

Question 5

Clinical features of cholangiocarcinoma

Answer 5

Jaundice and signs of biliary obstruction without alternative explanation

Question 6

Tumor markers for cholangio

Answer 6

CEA
CA 19-9

Question 7

Staging

Answer 7

• MDCT abdomen/pelvis
  +/- MRCP
• CT chest w/ contrast
• PET if no evidence of distant mets

Question 8

Early stage cholangio mgmt

Answer 8

• Surgery is curative but few patients present early for surgery to be an option

Question 9

management of obstructive jaundice

Answer 9

biliary decompression

Question 10

Surgical management of locoregional distal cholangiocarcinoma

Answer 10

pancreaticoduodenectomy (Whipple procedure)

Question 11

Sugical management of intrahepatic cholangiocarcinmoa

Answer 11

Hepatic resection

Question 12

Management of node positive loco-regional disease

Answer 12

Chemoradiotherapy

Question 13

mets beyond what region contraindicate surgical resection

Answer 13

Beyond the porta hepatis (

Question 14

Most common actionable mutations in cholangio

Answer 14

• IDH1 - R132C
• FGFR2 translocations

Question 15

First line based on TOPAZ 1

Answer 15

gem, cis, durvalumab

Question 16

Adjuvant management of resected biliary tract cancer + trial

Answer 16

Capecitabine (BILCAP trial)

Question 17

Preferred second line

Answer 17

• targeted therapy if actionable mutation
• pembro if MSI-H
• FOLFOX if none of the above

Question 18

IHC pattern of cholangiocarcinoma

Answer 18

CK7 + CK19 positive, CK20 negative

Question 19

Ivosidenib approved for what line of therapy

Answer 19

Sevond line