Spontaneous Rupture of a Fibrolamellar Variant Hepatocellular Carcinoma in an Innocent Liver

Abstract

Introduction: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a unique variant of hepatocellular carcinoma. The majority of cases present with nonspecific symptoms like vague abdominal pain, weight loss and fatigue. Ruptured FL-HCC occurs rarely and mortality in the acute phase is very high. We report a rare case of a ruptured FL-HCC successfully managed with transarterial embolization for hemostasis. Case: A 37-year-old male previously in good health presented with a severe, sharp epigastric pain that started one hour prior to the presentation. He denied trauma, fever, nausea, vomiting, diarrhea. Tenderness in the epigastrium was noted, with no palpable masses, guarding or rigidity. His blood pressure and pulse were 159/105 mmHg and 105 beats/min. Platelets and coagulation parameters were within normal limits, while hemoglobin of 12 g/dL, wbc of 11 k/mL and elevated transaminases (AST of 214 IU/L, ALT of 138 IU/L, ALP 98 IU/L, T.Billi 1.5 mg/dL) were noted. Abdominal CT scan with contrast revealed an 8 cm lobulated mass with central hypodensity in the left hepatic lobe with perilesional blood and free pelvic fluid, indicating tumor rupture (figure 1). CT angiography showed tumor neovascularization from a branch of the left hepatic artery which was embolized using transarterial gelfoam. Liver MRI (figure 2) and biopsy (figure 3) were consistent with fibrolamellar variant hepatocellular carcinoma. Hepatitis serologies and AFP levels were unremarkable. After 4 days, as the symptoms resolved and the lab results were stable, patient was discharged and underwent a left hepatectomy 3 weeks later. Discussion: FL-HCC occurs commonly in the left lobe of a young and non-cirrhotic liver. Typically cross sectional imaging reveals a lobulated mass with well-defined margins, areas of hypervascularity and a central calcified scar. Histologic appearance is characterized by eosinophilic polygonal shaped cells separated by lamellar fibrosis. Surgical resection is the treatment of choice with better outcome when compared to conventional HCC. Disease recurrence after complete surgical resection is however high in the first 5 years. Tumors > 5 cm in size are at high risk for rupture with high mortality and recurrence rates secondary to significant spillage of tumor. While an emergency hepatectomy is preferred in unstable patients, those that are hemodynamically stable can undergo radiologic transarterial embolization for hemostasis followed by staged hepatectomy.Figure: Axial section of CT Abdomen and Pelvis with IV contrast: (on the left) An 8 x 7 cm heterogeneous lobulated mass with central hypo density in the left hepatic lobe with arrow pointing perilesional blood concerning for tumor rupture. (on the right) An arrow pointing hyperdensity fluid surrounding urinary bladder likely blood from tumor rupture.Figure: MRI of the abdomen with liver protocol post embolization showing a 7.7 x 6.6 cm well circumscribed expansile lesion in left lobe of liver with global hypo T2 signal intensity with areas of hyper T2 signal intensities as well as central stellate shaped scar of low T2 signal intensity.Figure: Histopathologic examination of haematoxylin and eosin stained liver biopsy specimen (on the left) under high power magnification showing tumor cells with hepatocellular appearance: large polygonal cells, ample eosinophilic cytoplasm, with focal bile pigment, marked nuclear atypia, and Intranuclear pseudoinclusions. (on the right) under low power magnification showing tumor cells separated by hyaline collagen.