Case Presentation:
A 26 year old male with past medical history significant for gastroesophageal reflux disease, presented with complaints of ongoing right upper quadrant and epigastric abdominal pain over the previous 6 months. He reported 40-50 lbs weight loss over 6 months. He did not report any family history of liver cancers. His physical examination was only remarkable for tenderness in RUQ and epigastric region. Routine laboratory tests revealed unremarkable serum chemistry, blood counts and coagulation profile. Abdominal Ultrasonography and computed tomography were significant for large, 12cmx8cmx7cm, enhancing mass in left hepatic lobe and with tumor invading the left portal vein with expansion of the Vein. Tests for infectious hepatitis, hemochromatosis, and tumor markers were negative. Percutaneous biopsy showed moderately differentiated fibrolamellar cancer of liver. He underwent extended Left hepatectomy with tumor ablation. There was no involvement of Lymph nodes on surgical dissection. His post – op course was unremarkable.
Discussion:
Fibrolamellar carcinoma is a rare primary liver tumor that occurs in adolescents and young adults and is not associated with underlying liver disease. Since it is amenable to aggressive surgical treatment, early diagnosis is imperative. It has significantly better survival than HCC associated with cirrhosis. We report a case of Fibrolamellar Hepatocellular carcinoma with portal vein invasion. In this case, the patient presented with a common complaint of abdominal pain but was found to have this rare diagnosis. The underlying cause of fibrolamellar carcinoma remains unknown and it appears to be different from that of hepatocellular carcinoma, cholangiocarcinoma, and hepatoblastoma. Surgery is the mainstay of treatment for patients with fibrolamellar carcinoma. Primary tumor resection followed by surveillance for relapse offers best overall survival in these patients.
Conclusions:
Fibrolamellar carcinoma most often presents as an abdominal mass or with vague signs and symptoms such as abdominal pain, weight loss, and/or malaise. Since it is a rare tumor, it may not be considered in the differential. It should be suspected in young patients with liver mass and no underlying liver disease.