Contributed by Randall G. Lee, M.D.
PATIENT HISTORY:
Per referral letter, the patient is a middle aged male who has had fluctuating liver function tests. His workup by blood tests, including serologic tests, has been unrevealing. Clinically, at times, he has presented with jaundice. There is a slight clinical suspicion for primary sclerosing cholangitis, but imaging studies have not been performed. Review of outside material.

Final Diagnosis (Case 91)

LIVER, NEEDLE BIOPSY -
  1. FRAGMENTED SPECIMEN WITH ARCHITECTURAL ALTERATIONS SUGGESTING EVOLVING CIRRHOSIS.
  2. BILIARY FIBROSIS WITH CHRONIC BILE DUCT INJURY AND CHANGES OF CHRONIC CHOLESTASIS (See comment).

Comment:
The features in the biopsy point strongly to a chronic biliary disorder that has evolved with bridging fibrosis and early cirrhosis. No diagnostic bile duct lesions are present, but given the duct atrophy and only modest inflammatory component, the most likely possibility is primary sclerosing cholangitis or another form of low-grade biliary obstruction such as a duct stricture. Drug-induced injury cannot be excluded, and primary biliary cirrhosis and autoimmune cholangitis are less likely possibilities. Cholangiography should be helpful in clarifying the nature and site of the obstruction; if the large ducts are found to be uninvolved, a diagnosis of so-called small duct primary sclerosing cholangitis should be considered. Previous Biopsies on this Patient:
None

TPIS Related Resources:
Liver Transplant Topics

Gross Description - Case 91

The specimen consists of four (4) consult slides with an accompanying surgical pathology report.

Microscopic Description - Case 91

The liver biopsy is fragmented with zones of fibrosis focally present along their periphery. The parenchyma shows evidence of hepatic plate thickening and architectural irregularities. Although not definitive, this combination of changes suggests an evolving cirrhosis. The fibrous zones show focal ductular proliferation with duct atrophy, characterized by shrunken, disproportionately small ducts with nuclear hyperchromasia and irregularity, together with probable duct loss. The peripheries of the fibrous septa show loose, edematous connective tissue with intermingling of a sparse inflammatory infiltrate into the adjacent parenchyma, features indicative of a chronic cholestatic condition. Although no florid duct lesions or periductal obliterative fibrosis are seen, these changes along with the bile duct alterations point to a chronic biliary process. Because of the relative sparseness of inflammation within the fibrous septa, a diagnosis of primary sclerosing cholangitis is favored. However, similar appearances could be produced by other forms of low-grade biliary obstruction, by drug-induced duct injury, or less likely, by primary biliary cirrhosis or autoimmune cholangitis.

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