A red flag and a red herring-dual pathology of isolated polycystic liver disease with underlying epithelioid angiosarcoma resulting in recurrent hepatic cyst haemorrhage

Polycystic liver disease (PLD) is an uncommon inherited condition that leads to progressive development of hepatic cysts that can become complicated by rupture, infection, and bleeding. We detail the case of a 53-year-old female presenting with haemoperitoneum secondary to hepatic cyst bleeding on a background of isolated polycystic liver disease. Despite extensive investigations to localize a bleeding source, repeated angioembolisation, and partial hepatic resection, the patient had recurrent hepatic haemorrhage. Posthumous histology of the resected liver revealed malignant epithelioid angiosarcoma, a rare vascular tumour underlying the PLD. This is the first reported case with a dual pathology of polycystic liver disease and primary liver epithelioid angiosarcoma. Infiltrative malignancies should be considered in atypical cases of recurrent hepatic haemorrhage.