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A 52-year-old male patient
began to suffer from progressive yellowish discoloration of
skin and dark urine since 1 month ago. The brown color of his
stool became lighter in 2 weeks. Generalized skin itching
developed for 3 days. Mild anorexia and weight loss for 2 kg
was also noted in this month. There was no abdominal pain or
fever developed during this period. He did not smoke and drank
alcohol only on social occasions. He denied any history of
chronic liver disease, parasite infestation or chronic drug
usage.
Physically, his
consciousness was clear, vital signs were normal. Skin was
yellowish discolored, but there was no obvious scratch mark.
Sclera was deeply icteric. Conjunctiva was not pale. No spider
angiomata or gynecomastia was noted. Cardio-pulmonary
examinations were unremarkable. Abdomen was soft and flat, and
there was no tenderness. Liver, gallbladder and spleen were
not palpable. There was no shifting dullness. Extremities were
freely movable and without pitting edema. On digital
examination of rectum, no tumor was felt. The color of stool
was light brown. No blood or mucus was stuck on the glove.
On admission, his white blood cell count was 7.4 K/μL, hemoglobin
13.4 gm/dL, platelet count 121 K/μL, and prothrombin time 12.8
sec (control 11.2 sec). Blood chemistry revealed normal blood
urea nitrogen (18 mg/dL), creatinine (0.9 mg/dL), fasting
blood sugar (97 mg/dL) and cholesterol (198 mg/dL). But liver
tests showed serum albumin 3.4 gm/dL, globulin 3.5 gm/dL,
total bilirubin 28.7 mg/dL, direct bilirubin 19.5 mg/dL,
aspartate aminotransferase (AST) 89 U/L, alanine
aminotransferase (ALT) 102 U/L, alkaline phosphatase (ALP) 972
U/L (normal < 220 U/L), gamma glutamyl transpeptidase
(g
-GT) 582 U/L (normal <
52 U/L). Hepatitis B surface antigen (HBsAg) and
anti-hepatitis C antibody (Anti-HCV) were negative. Serum alpha-fetoprotein (AFP) was
below 20 ng/ml, but carcino-embryonic antigen (CEA) was
mildly elevated to 8.7 ng/ml. Urine bilirubin was
positive, but urobilinogen was negative. No occult blood was shown in stool.
The
initial impression was "obstructive jaundice". Immediate
abdominal ultrasound (Fig.1)disclosed dilated bilateral ducts with a small hypoechoic nodule at
hilum, but normal size of gallbladder and common bile duct (CBD). No stone can be identified in
the intra- or extra-hepatic ducts. The width of main pancreatic duct was also normal and pancreas was
unremarkable. Bilateral percutaneous transhepatic cholangiography with drainage (PTCD) was performed.
The cholangiogram (Fig. 2) revealed dilated bilateral intra-hepatic ducts with stenotic area around the
bifurcation of right and left hepatic duct. The common hepatic duct (CHD), gallbladder,and CBD were free of
tumor and normal in size. No filling defect could be identified in the whole biliary system. Because the
stenotic area was so narrow that the drainage catheter could not pass through, the bile could only be drained
externally. A cytological examination of bile obtained from the drainage bag disclosed only degenerative
cells. Unfortunately, sepsis developed after the PTCD procedure. Despite strong antibiotic therapy, he passed
away 10 days later. An autopsy was performed. Grossly, the liver was greenish. On cutting, there was a small
(about 3×2 cm) whitish, ill-defined tumor encasing left hepatic duct after bifurcation and extending to the
right hepatic duct (Fig. 3). Invasion of whitish tumor into surrounding liver parenchyma was evident.
Microscopically, it showed adenocarcinoma in a desmoplastic stroma (Fig.4).
 病情解說
這個52歲男性病人以前並無肝膽方面病史，一個月前開始注意到皮膚變黃，而且越來越嚴重，小便顏色也漸漸變深，呈現可樂的顏色。住院前幾天大便顏色開始變淡成為淺黃色。住院後的初步檢查指向有阻塞性黃疸的可能，而且為了盡快緩解病人的黃疸，於是為病人作了經皮穿肝膽管攝影術併膽汁引流（PTCD）。發病時期並無發燒或上腹痛的病史，而且平常亦不曾有併發黃疸、上腹痛、發燒的經驗，因此不像是膽道結石引起的阻塞性黃疸。膽管攝影顯示肝門區左右肝管匯合處有狹窄現象，而且造成兩邊肝管擴張，但是匯合處下方的總肝管、總膽管、及膽囊都沒擴張，因此判斷是Proximal
bile duct tumor （又稱hilar cholangiocarcinoma或Klatskin
tumor）。不幸病人最後死於敗血症。
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Case Discussion