A 51-year-old woman presented with jaundice, pruritus, incessant abdominal pain that radiated to the back, and postprandial nonbilious vomiting. She had undergone 8 months of chemotherapy for pancreatic carcinoma. Her past medical history was significant for ulcerative colitis (UC) diagnosed in 1974. She had a total proctocolectomy with construction of a Koch pouch in 1981
Prepared by Daniel B. Brown, MD; Craig B. Glaiberman, MD;
Anthony W. Allen, MD; Harjit Singh, MD; and Peter N. Waybill,
MD, of the Department of Radiology, Pennsylvania University
Hospital, Hershey, PA.
A 51-year-old woman presented with jaundice, pruritus, incessant
abdominal pain that radiated to the back, and postprandial
nonbilious vomiting. She had undergone 8 months of chemotherapy for
pancreatic carcinoma. Her past medical history was significant for
ulcerative colitis (UC) diagnosed in 1974. She had a total
proctocolectomy with construction of a Koch pouch in 1981.
She was admitted for liver failure. Admission lab work revealed
a total bilirubin of 20.4 mg/dL (normal 0.1-1.2 mg/dL) and an
alkaline phosphatase of 2620 U/L (normal 40-130 U/L). A CT scan
(figure 1) showed gross ascites, a 4 * 4 cm pancreatic mass which
invaded the lesser sac and occluded the portal vein, massive
abdominal varices, extensive retro-peritoneal adenopathy, and a
common bile duct that was 2.1 cm in diameter. Suspected bilateral
deep venous thromboses were confirmed by ultrasound.
The patient was referred for percutaneous trans-hepatic
cholangiography (PTHC) and possible percutaneous biliary drainage.
At PTHC, the intrahepatic biliary system was noted to be irregular
and had a beaded appearance (figure 2). The patient's condition
deteriorated quickly over the next few days and she died shortly
Metastatic adenocarcinoma of the pancreas
At autopsy, a large hemorrhagic and necrotic mass in the head of
the pancreas was present, consistent with pancreatic
adenocarcinoma. There was extensive infiltration of the porta
hepatis. Intrahepatically, microscopic foci of pancreatic carcinoma
diffusely surrounded and encased the portal triads including the
periductal regions. There was no evidence of periductal fibrosis,
inflam-mation, or duct obliteration typical of primary sclerosing
cholangitis (PSC). The final diagnosis was metastatic
adenocarcinorna of the pancreas infiltrating the intrahepatic
biliary system, mimicking PSC.
Approximately 80% of pancreatic adenocar-cinomas occur in the
head of the gland.
The mean age of onset is 55 years and the disease has approximately
a 2:1 male:female incidence. Patients typically present late in the
course of the disease with weight loss and abdominal pain that
radiates to the back. At the time of diagnosis, up to 90% of cases
show local invasion and/or metastases to the liver and/or lung.
Spread along the biliary tree producing diffuse narrowing of the
intrahepatic biliary radicals is rare.
Less than 2% of patients diagnosed with pan-creatic
adenocarcinoma survive 5 years after diagnosis.
Even if "curative" resection (pancreaticoduodenectomy) is
performed, 5-year survival only approaches 10%. Multiple
combinations of chemotherapy and radiation have been tried with
little improvement in survival. Metal stents may be placed to
palliate non-operative disease. Splanchnic blocks can also be used
to control intractable pain.
Metastatic adenocarcinoma of the pancreas simulating sclerosing
cholangitis at cholan-giography has been described.
As in our patient, the extrahepatic ducts were dilated, a finding
not typical of PSC. Pancreatic cancer most often causes diffuse
biliary dilation proximal to a distal obstruction. Although most
patients with sclerosing cholangitis have involvement of the
extrahepatic ducts, the diagnosis cannot be excluded if they do not
appear affected at cholangiography.
This suggests disease progres-sion from within the liver distally
to involve the extrahepatic ducts.
PSC is a cholestatic syndrome of unknown etiology involving both
the intra- and extrahepatic ducts.
PSC is most common in young men and presents insidiously with
pruritus, jaundice, and hepatosplenomegaly. Pathologic hallmarks
include periductal fibrosis and inflammation resulting in scarring
of the biliary tree. Approximately 50% to 75% of patients have one
or more signs of liver disease at the time of diagnosis of PSC.
Elevation of alkaline phosphatase, aspartate transaminase, and
bilirubin levels are variable. Cirrhosis, portal hypertension,
varices, and ascites result as PSC progresses to overt liver
failure. Up to 10% to 15% of cases of PSC progress to
cholangiocarcinoma. Differentiation of PSC and cholangiocarcinoma
is difficult and stricture progression, polyp formation, or ductal
dilation identified during interval cholangiography must be
considered suspicious for malignant change.
Definitive diagnosis can be made with biopsy via percutaneous
access or endoscopic retrograde cho-langiopancreatography (ERCP).
Today, PSC continues to be one of the leading indications for liver
Toxins, certain viruses (CMV and Reovirus type III), ischemia,
AIDS, and presence of HLA-B8 and HLA-DR3 have all been suspected of
A patient expressing both HLA-B8 and HLA-DR3 has an estimated
10-fold increased relative risk of developing PSC, suggesting
genetic and immunologic mediation of disease abnormality.
Disease processes associated with PSC include UC, pancreatitis, and
Patients diagnosed with UC have a 10% chance of developing PSC.
In contrast, up to 70% of people with PSC have UC as well.
PSC may present a number of years before or after the diagnosis of
the bowel disease. PSC may remain undetected until liver failure
becomes fulminant. However, coexistent PSC in UC patients is
becoming recognized more frequently because of better diagnostic
procedures (ERCP and PTHC), screening blood work, and physician
awareness of the association of PSC with UC.
UC is bimodal, presenting in patients between the ages of 15 to
30 and 50 to 70 years. The colitis is a submucosal inflammatory
infiltration begin-ning in the rectosigmoid colon and extending
proximally in a continuous fashion. Crypt abscesses and mucosal
ulcerations are common and can involve the entire colon. Typically,
the ulcerations do not extend through all layers of the bowel wall
like the lesions of Crohn's disease. The stuttering clinical course
is fraught with attacks of varying intensity that are characterized
by crampy abdominal pain, frequent loose stools containing blood
and mucus, fever, and anorexia. Toxic megacolon, the most serious
complication, can lead to anemia, hypotension, perforation,
peri-tonitis, and septicemia.
Proctocolectomy is the definitive treatment for UC but does not
slow the progression of PSC.
Extra-colonic manifestations of UC include arthritis, ankylosing
spondylitis, sacroiliitis, uveitis, and aphthous ulcers, which can
wax and wane with flare-ups.
PSC can present clinically either before or after UC. Although
the findings at cholangiography are typical, they are not
pathognomonic. Subtle differences, such as the lack of extrahepatic
biliary stricture in this case should lead to the consideration of
other diagnoses. Review of physical findings can suggest the
diagnosis in most cases. Knowledge of all other pathologic
processes is essential to arrive at the correct diagnosis.
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