Secondary changes in the peripheral liver due to kaposiform haemangioendothelioma in the hepatic hilum: a report of two transplant cases.
Rishi Agrawal, Owen Lawrence Cain, James Ferguson et al.
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Case in brief
Two patients underwent liver transplantation for liver failure associated with clinically unsuspected kaposiform haemangioendothelioma (KHE) at the hepatic hilum. In one, liver failure beginning in childhood had been labelled cryptogenic cirrhosis; the explant showed hilar KHE surrounding portal-vein branches, with peripheral obliterative portal venopathy and nodular regenerative hyperplasia but no cirrhosis. The other had been diagnosed with primary sclerosing cholangitis (PSC) after obstructive liver failure and initial investigations. The explant revealed KHE around the common hepatic duct and peripheral biliary-pattern cirrhosis, supporting secondary sclerosing cholangitis. Transplantation provided the diagnosis in both cases.
What made this case unusual
An unsampled hilar KHE produced distinct secondary changes in the peripheral liver, mimicking cryptogenic cirrhosis in one patient and PSC in the other.
Diagnostic / clinical pearl
When peripheral liver biopsy findings do not fit the clinical history, consider whether an unsampled hilar lesion is causing secondary parenchymal changes.