Challenging HCC surveillance in a case with a history of peliosis hepatis and liver cirrhosis CHILD A due to a hypoplastic portal vein

Background:

Peliosis hepatis is a rare disease which is characterized by proliferation of sinusoidal hepatic vessels leading to blood-filled cystic cavities in the liver tissue. The etiology is unknown. Risk factors are various medicines (e.g. steroids, Azathioprin), a history of infectious diseases (e.g. HIV, Bartonella henselae, tuberculosis) or immunodeficiency/-suppression. In most cases peliosis is an incidental finding because the majority of the patients remain asymptomatic. Few patients present with abdominal pain, jaundice, hepatomegaly, portal hypertension with its complications and liver failure in worst case.

Case report:

In 2003 at the age of 14, our male patient presented with jaundice and gynecomastia. A liver cirrhosis CHILD A due to hypoplasia of the portal vein was diagnosed. Because of portal hypertension several ligations of esophageal varices were performed in the last thirteen years. Liver function remained stable. Every six months an ultrasound was performed for hepatocellular carcinoma (HCC) surveillance. In 2012 at the age of 23, two hepatic lesions were found in liver segment VII and VIII by ultrasound, each about 3 cm in diameter. Their contrast behavior showed hyperperfusion without signs of late wash-out. MRI scan revealed further lesions compatible to HCC DD cholangiocarcinoma (CCC). Biopsy of the suspect lesions showed a prominent peliosis hepatis without signs for malignancy. An etiology could not be found. One year later another biopsy was performed because lesions showed progress in diameter. Once again malignancy was ruled out and the diagnosis was confirmed. Until now peliosis hepatis shows no progress in annually ultrasound and no hepatocellular carcinoma could be detected in annually MRI scans.

Conclusion:

The presented case describes a long-term course of a patient with peliosis hepatis. It shows the difficulty of diagnosing peliosis hepatis and differentiating it from malignant tumors only by imaging. This leads to challenging long-term follow ups, especially in patients with additional liver cirrhosis and high risk of HCC. Close monitoring is required. Finally, diagnosis can be made only by biopsy and histopathological examination.