Fibrolamellar hepatocellular carcinoma presenting with early recurrent thrombosis

Fibrolamellar hepatocellular carcinoma (FL-HCC) was first described by Edmonson in 1956 as an adult type of liver tumour in a 14-year-old female with no underlying liver disease [1]. Despite FL-HCC being a variant of hepatocellular carcinoma (HCC), it differs from HCC in that FL-HCC most commonly affects younger patients (10–35 years) without underlying liver disease. FL-HCC patients are more likely to be female [2]. FL-HCC often presents with abdominal pain, nausea, abdominal fullness, malaise, and weight loss. Rarely, patients present with symptoms related to tumoural hormone production, such as gynaecomastia resulting from aromatization of androgens, or paraneoplastic production of thyroid hormones or -human chorionic gonadotropin (-hCG) [3, 4].
A 32-year-old female patient was admitted to the internal medicine service with complaints of recurrent deep vein thrombosis, left leg/left arm thrombosis, and pulmonary embolism with pancytopenia. Due to thromboembolism, the diameter of her left leg was larger, and anticoagulant treatment was started. Blood examination revealed elevated D-dimer (1612 ng/dl) and normal bilirubin, prothrombin time, thrombocyte, and -fetoprotein. Collagen tissue diseases were suspected in this young female patient due to recurrent thromboembolism. Anti-nuclear antibodies (ANA), paroxysmal nocturnal haemoglobinuria (PNH) panel, the von Willebrand factor cleaving protease ADAMTS13, and thrombophilia panel were negative. Phospholipid antibodies were negative, antithrombin-3, protein C, and protein S were within normal range, but lupus anticoagulants (LA) were positive. LA were retested for the control, and this time they were negative. In addition, the level of vitamin B12 in the blood was 84 pg/ml, which is below the normal range (157–883 pg/ml). Because collagen tissue diseases and hereditary thrombophilia were ruled out, an underlying malignancy was suspected, due to the absence of collagen tissue diseases and hereditary thrombophilia. Abdominal CT (Figures 1 A, B) and MRI (Figures 1 C, D) were performed. A 100  82  54 mm lobule heterogeneous mass with nodular calcifications from segments 2 to 3 was detected in the left liver lobe. Fine needle aspiration biopsy was performed with EUS. The biopsy showed polygonal eosinophilic cells. Their cytoplasm was densely granular or foamy with the presence of granules and pale bodies. Tumour cells were positive for arginase and hepatocyte paraffin-1 (HepPar-1); both proteins...


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