Secondary changes in the peripheral liver due to kaposiform haemangioendothelioma in the hepatic hilum: a report of two transplant cases
Agrawal, Rishi Cain, Owen Lawrence Ferguson, James Hartley, Jane Mateos, Rebeca Sanabria Bartlett, David C McGuirk, Simon P Brown, Rachel Mary
Agrawal, Rishi
Cain, Owen Lawrence
Ferguson, James
Hartley, Jane
Mateos, Rebeca Sanabria
Bartlett, David C
McGuirk, Simon P
Brown, Rachel Mary
Abstract
Aims: Kaposiform haemangioendothelioma (KHE) is a rare vascular neoplasm of infancy and childhood that typically affects the soft tissues of the extremities; however, a variety of visceral sites have been reported. When KHE is located within the liver hilum, it may involve hilar structures and lead to secondary changes in the hepatic parenchyma. We report the histological changes in the explant livers of two patients transplanted for liver failure secondary to clinically unsuspected KHE at the hepatic hilum.
Methods and results: Patient A initially presented with the signs and symptoms of liver failure at the age of 6. Despite extensive clinical work up, no aetiology was identified and she was diagnosed with 'cryptogenic cirrhosis'. The patient underwent liver transplantation at age 23, and her explanted liver revealed a vasoformative tumour within the hilum surrounding branches of the portal vein. Immunohistochemistry confirmed a diagnosis of KHE. Her peripheral liver showed features of obliterative portal venopathy and nodular regenerative hyperplasia. There was no evidence of cirrhosis. Patient B presented at age 7 with obstructive features of liver failure. Based on imaging studies and initial liver biopsies, a diagnosis of 'primary sclerosing cholangitis' (PSC) was rendered. However, liver transplantation at age 11 revealed KHE at the hepatic hilum surrounding the common hepatic duct. The peripheral liver demonstrated biliary-pattern cirrhosis with periductal fibrosis and ductular reaction, supporting a diagnosis of secondary sclerosing cholangitis rather than PSC.
Conclusions: In conclusion, changes seen in peripheral liver biopsies can sometimes be a consequence of unsampled lesions at the liver hilum. KHE is a very rare example of this, but this scenario should be considered when liver biopsy findings do not correlate well with the clinical history.
Affiliations
University Hospitals Birmingham NHS Foundation Trust; Birmingham Women's and Children's NHS Foundation Trust
Date
2026-03-28
Type
Article
Collections
Citation
Agrawal R, Cain OL, Ferguson J, Hartley J, Mateos RS, Bartlett DC, McGuirk SP, Brown RM. Secondary changes in the peripheral liver due to kaposiform haemangioendothelioma in the hepatic hilum: a report of two transplant cases. Histopathology. 2026 Mar 28. doi: 10.1111/his.70147. Epub ahead of print.