Liver PEComa. An extremely rare case report with complicated post-operative course

Abstract

Background: Perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal tumor with heterogeneous clinical behavior. Liver PEComas are very rare. Methods: The case of a 47-year-old man with hepatic PEComa and a complicated postoperative course is reported. Results: The patient had a history of systemic lupus erythematosus (SLE) and developed epigastric pain. An abdominal CT showed a large (7 cm) heterogeneous mass on segments I/IV, in direct contact with the inferior vena cava and portal vein. Percutaneous liver biopsy showed PEComa and the patient underwent left hepatectomy. On post-op day 7, he became pyrexial, with significant elevation in transaminases and inflammatory markers. Imaging (CT/MRI) showed a hypodense area in segment VIII close to resection margin. Blood cultures were taken and broad spectrum iv antibiotics were administered. As he continue to be pyrexial with a clinical picture of sepsis he underwent a second laparotomy, where necrosis of segment VIII was found and it was resected. As he became again pyrexial (on day 7), a new CT abdo was done revealing concentric hepatic artery stenosis with hypodense areas on segment VI with sphenoid shape. Acute exacerbation of SLE with right hepatic artery partial thrombosis or vasculitis was considered to be the diagnosis. He was commenced on steroids and anticoagulants with good clinical response. Conclusion: Management of hepatic PEComas can be very challenging especially in patients with significant co-morbidities.