2. Brief info
• Patient: 詹 XX
• Sex: male
• Age: 63 years old
• No DM/HTN
• Alcohol(+): 高粱 or beer
• Smoking(-)
3. 10/17/2010 At ER
- Chief complain -
• Poor appetite for 2 weeks
• Generalized malaise
4. Present illness
• He has complained of poor appetite and
general malaise for 2 weeks, associated
with RUQ pain, tea color urine, clay
color stool passage and BW loss 2 kgs
in recent 2 weeks. So he sent to our ER
for help
5. Physical exam
• Consciousness: clear
• Head-- Conj: pink, Sclera: icteric(+) , Pupil: isocoric, Light reflex: +/
+
• Ear, Nose: normal
• Oral cavity: normal
• Neck-- Supple
• Thyroid: normal
• Jugular vein: not engorged
• Chest-- Heart sound: regular without Murmur
• Breath sound: clear
• Abdomen-- flat, normoactive bowel sound, Tenderness over RUQ
• Liver and spleen: not palpable
• Lymphadenopathy ( Neck, Axilla, Inguinal ): not palpable
• Genital organs: normal
• Spine & Extremities: no deformities, no edema
• Peripheral Vascular System: normal
• Neurological System: full & equal
6. Lab data at ER
• DBIL/TBIL=0.73
• >70% - conjugated
hyperbilirubinemia
• Most likely due to
hepatobiliary tract
disease
7. 10/17/2012
Abdominal CT with contrast
• Suspect klatskin tumor with bilateral IHD
dilatation.
• Right renal cyst.
• Sigmoid diverticulitis.
12. 10/18/2012
Cholangiography
• The PTCD catheter traverses the hilum
from the right IHD towards the left IHD.
• The left IHD was not demonstrated,
recommend revision.
15. 10/17/2012
At ER
-EKG
-CXR
-KUB
-Abd CT
10/18/2012
-Abd echo
-PTBD
Admission
-Cholangiography
-MRI
-Check tumor
markers
and hepatitis
markers
10/21/2012
-f/u LFT + TBIL
10/23/2012
-PTCD revision
10/26/2012
-Consult
GS for OP
evaluation
10/27/2012
-Cardiac echo
10/29/2012
-Roux-en-Y
Hepaticojejuostomy
-transfer to AICU
after OP
11/01/2012
-transfer to ward
11/02/2012
-patho report
11/03/2012
-try liquid diet
-pass flatus
11/04/2012
-stool passage
11/06/2012
-try soft diet
11/12/2012
-Discharge home
16. Cholangiocarcinoma
• Intrahepatic type (10%)
• Perihialr type (60~80% )
– Also called Klatskin tumor
– Named after Dr. Gerald Klatskin
– In 1965 described local, sclerosing
adenocarcinomas of the hepatic fork as
clinically independent tumor entities
• Extrahepatic type (10~30%)
17. Staging systems for hilar CC
• Bismuth-Corlette classification
• American Joint Committee on Cancer
(AJCC) TNM staging system
18. Epidemiology
• 5th most common tumor of the GI tract
• Western countries, 1-2 cases per 100,000
• Northeastern Asia, 96 cases per 100,000
• About 1,500 case of newly diagnosed klatskin tumor
annually in the United States
• About 1800 men and 4200 women in Germany develop
gall bladder or extrahepatic cholangiocarcinomas every
year
• 1.3~2.6% of annual cancer-related deaths worldwide
• Multiple studies have documented a steady increase in
the incidence of cholangiocarcinoma over the past
several decades
19. Clinical symptoms
• Symptoms usually appeared when tumor
causes obstruction of biliary tract and causes
painless jaundice
• pruritis (66%)
• RUQ pain (40~50%)
• Weight loss (40~50%)
• Fever (20%)
• Clay stool
• Tea color urine
21. Diagnosis
• CA 19-9 not specific for cholangiocarcinoma
• Use of CEA and CA 19-9 as markers has been
suggested when values are greater than 5.2 ng/ml
and 180U/ml respectively since the sensitivity is
100% and specificity is 78%.
• Abdominal USG – IHD/CHD dilation
• Abdominal CT – Bilobar ductal dilation with a
contracted gallbladder or disjunction of the hepatic
lobes, with or without thickening, suggests a
Klatskin tumor
22. • MRI/MRCP - favored over CT
• Cholangiography – for tissue diagnosis
or therapeutic decompression/drainage
• PET scan - but less useful for infiltrating
lesions
• Brush cytology and biopsy specimens
24. Inportance of Surgery Methods
• The hilar CC has an invasive growth pattern and
an extensive subepithelial spread which
emphasized the importance of confirmation of
negative margins by a frozen section during the
resection.
• The mean distance of the microscopic invasion
beyond the gross margin toward the liver was
found to be 16.8mm, which underlines the
importance of wider resections.
25. Surgical outcomes
• Intraoperative mortality in older studies was
between 7.5% and 18%
• Four newer publications have reported the
intraoperative death rates of 0% and 1.3%
• Five year survival rate that included patient
whose surgery did not conforrm to today’s
radical principles are 12.5~32.8%
• Newer studies with exclusively radically
operated and/or R0 resection yielded 5 year
survival of 35~44%, and even 72%
26.
27. Prognosis
• Only 10% life expectancies of 5 years
• Surgery is the only feasible option
• 90% of distal cholangiocarcinoma require
surgery
• Hilar type in about 56% need resection
28. American criteria for resection
• Absence of tumor involvement in the
retropancreatic lymph nodes and the
celiac artery and absence of distant
metastases
• No invasion of the portal vein of the main
hepatic artery (even though some
institution perform vascular reconstruction
in these cases)
• Absence of invasion in neighboring organs
• No disseminated disease
29. Chemotherapy
• Between 60~90% not suitable for resection at
the moment of diagnosis.
• Life expectancy with inoperative disease – only
a few months
• National Comprehensive Cancer Network
suggest:
– Extrahepatic CC: Fluorophyrimidine or gemcitabine
based chemotherapy
– Intrahepatic CC: Fluorophyrimidine or gemcitabine
based chemotherapy with ablative therapy (radio
frequncy, cryotherapy or microwave ablation).
30. References
• Helmut Witzigmann, Marcus Wiedmann, Christian
Wittekind, Joachim Mossner, Johann Hauss:
Therapeutical concepts and results for Klatskin tumors.
Deutsches Arzteblatt International 2008;105(9): 156-61
• Ljiljana Somer, Bojana Andrejic, Pavle Milosevic: Origin
and pathological characteristics of Klatskin tumor: a case
report and literature review. Pol J. Pathol 2012; 1:65-70
• Rodrigo Castano Llano, MD: Hilar cholangiocarcinoma
(Klatskin tumor). 2011 Asociaciones Colombianas de
Gastroenterologia, Endoscopia digestiva, Coloproctologia
y Hepatologia
• Johan Fevery: Review article Bilirubin in clinical practice:
a review. Liver international ISSN 1478-3223
Bismth-Corlette classification - Based on the biliary ducts involvemetn by the tumor. Type I – tumors below the confluence of the left and right hepatic duct Type II – tumors reaching the confluence Type IIIa/b – tumors involving the common hepatic duct and spreading to either the right or the left hepatic duct Type IV – tumors involving the confluence and both the right and left hepatic ducts AJCC TNM staging system - Based on the pathological criteria
Parasitic disease – Clonorchis sinensis Opistorchis viverrini Toxin – thorotraast (radio-contrast used in 60s) Lynch syn – auto dominant dz, increased risk of colon, ovarian, endometrial, brain, skin and cholangio CA Biliary papillomatosis – 83% related with cholangiocarcinoma Chronic liver disease – ex HBV, HCV cirrhosis Japan -> HCV increase 3.5 times over 10 year period US -> HCV 2.55 times greater risk Danish -> cirrhosis increases 10 times risk for choloangioCA HIV – up to 6.4 tims greater
MRI has sensitivity of 94% and specificity of 100% to assess the Bismuth-Corlette classification Most surgeons request cholangiography to assess the resectablility of the tumor In 40~70% of patients with suspected extrhepatic cholangiocarcinoma is confirmed by combination of brush cytology and clamp biopsy.
