Table 2.
Benign lesions mimicking a perihilar cholangiocarcinoma: clinical differential diagnostic criteria.
| Primary Sclerosing Cholangitis | Lymphoplasmacytic Sclerosing Cholangitis-Sclerosing Cholangitis IgG4 Positive (as Part of Systemic IgG4-Related Disease) | Recurrent Pyogenic Cholangitis | Inflammatory Pseudotumor (IPT) |
Impacted Stones with Periductal Fibrosis | Mirizzi Syndrome | Portal Biliopathy | |
|---|---|---|---|---|---|---|---|
| Clinincal presentation | Jaundice, pruritus, abdominal pain. | Jaundice, pruritus, abdominal pain. | Jaundice, fever, abdominal pain. | Asympthomatic or abdominal pain and fever. | Jaundice, fever, abdominal pain. | Jaundice, ± fever, abdominal pain. | Often asympthomatic. Patient history of extrahepatic portal vein thrombosis is the most common cause of portal biliopathy; rare in cirrhosis, portal vein fibrosis without cirrhosis and congenital hepatic fibrosis. |
| Epidemiology | Age at diagnosis 44.2 y.o. ± 17.4 (11–81) *. The age-adjusted incidence rate for males was numerically greater than females. Patients are usually nonsmokers, and about 2/3 have a coexistent IBD (75% ulcerative colitis). |
Middle to upper age, with an onset at 50–70 years, Male:female ratio = 3:7. |
More frequent between the third and fifth decades. | Incidence correlates to gallbladder stones. | |||
| Laboratory test | Elevated ALP, GGT, bilirubin. Different non-specific autoantibodies correlate with PSC, such as P-ANCA, ANA, anti-smooth muscle autoantibodies |
Elevated serum IgG4 concentration (≥135 mg/dL). CA19-9 can be elevated. |
Elevated inflammatory markers (including erythrocyte sedimentation rate, C-reactive protein, and leukocyte count) are common. CA19-9 usually normal. | Elevated ALP, GGT, bilirubin. ± elevated inflammatory markers. CA19-9 can be elevated because of jaundice. |
Elevated ALP, GGT, bilirubin, GOT, GPT. CA19-9 can be elevated. |
||
|
Appropriate
imaging modality and features |
MRCP, ERC. Beaded appearance, pruned tree appearance, and band-like stricture. |
MRCP. Diffuse or segmental narrowing of the intrahepatic and/or extrahepatic bile duct, associated with the thickening of the bile duct wall. |
MRCP. Intraductal calculi and bile duct strictures. |
CT-scan, MRI. The CT-scan: lesions with variable c.e., may present as hypovascular with delayed enhancement because of fibrosis. The MRI may produce hypointense on T1 sequences with moderate-to-high hyperintense on T2 sequences. |
CT scan (scarce sensitivity for non-calcific stones) and MRCP. | CT scan, MRCP. MRCP most accurate, shows an extrinsic narrowing of the common hepatic duct, a gallstone in the cystic duct, dilation of the intrahepatic and common hepatic ducts, with a normal common bile duct. |
CT scan and portal MR and MRCP. Show portal cavernoma, paracholedochal and/or epicholedochal dilations, portosystemic shunts and abnormal morphology of the bile duct. |
| Specific investigation | Cholangiography, liver biopsy for doubtful cases. | Elevated serum IgG4 concentration (≥135 mg/dL). | Exclusion diagnosis. Percutaneous liver biopsy. | ||||
| Histo-pathological examination |
Obliterative, non-suppurative cholangitis with substantial periductular fibrosis, referred to as “onion-skin fibrosis”. |
Marked lymphocytic and plasmacytic infiltration and fibrosis. Infiltration of IgG4-positive plasma cells (>10 cells per high-power field). Storiform fibrosis. Obliterative phlebitis. |
Chronic, recurrent infections from parasites predispose to the development of pigmented calculi, cholangitic abscesses, and inflammatory strictures. | Inflammatory infiltrate consisting of lymphocytes, plasma cells, and histiocytes admixed with a variable proportion of fibroblasts and myofibroblasts. |
Acute or chronic cholecystitis. | ||
| Specific medical intervention | High dose of UDCA (20 mg/kg/d) improve liver function tests. | Prednisone 20–40 mg/d. | Improvement after antibiotics or after steroid administration. | Treat underlying disease. |
* Expressed as mean ± standard deviation (range). Abbreviations: ERC, endoscopic retrograde cholangiography; MRCP, magnetic resonance cholangiopancreatography; MRI, magnetic resonance imaging; CT scan, computed tomography scan; IBD, Inflammatory Bowel Disease; ALP, alkaline phosphatase; GGT, gamma glutamyltransferase; GOT, glutamic oxaloacetic transaminase; GPT, glutamic pyruvic transaminase; c.e., contrast enhancement; UDCA, ursodeoxycholic acid; P-ANCA, perinuclear antineutrophil cytoplasmic antibodies; ANA, antinuclear antibodies.