Fibrolamellar Carcinoma of the Liver with Vertebral Metastasis: A Rare Presentation

Abstract

Background: Fibrolamellar carcinoma is a very uncommon variant of hepatocellular carcinoma, (HCC) occurring in 0.6%-8.6% of all hepatocellular carcinoma cases. Unlike HCC, it usually occurs in younger individuals in their 2nd or 3rd decade of life without any underlying liver disease. Usual cases of metastasis include lymph node, lung, and peritoneum. We report a unique case of fibrolamellar carcinoma with metastasis to the lumbar spine. Case Report: This a 27-year-old Hispanic male with no significant past medical history, who presented to the ED with epigastric and lower back pain for one month. Pain was intermittent, non-radiating, and rated as 10/10 in severity. He admitted to a 10-15 lb weight loss in the preceding few months, with associated loss of appetite. He denied any nausea, vomiting, fevers, or chills. An abdominal US showed a heterogeneous echogenic mass in right lobe of the liver. A CT scan of the abdomen and pelvis revealed an ovoid enhancing right hepatic lesion with extension into the retroperitoneum. It involved the right hemidiaphragm and lungs, along with extensive lymphadenopathy, left hydronephrosis, and a lytic L4 lesion. Liver function tests and AFP were within normal limits, and hepatitis panel was negative. A CT-guided liver biopsy was obtained, which revealed hepatocellular carcinoma, fibrolamellar variant. Due to extensive disease, surgical resection was not possible, and therefore, chemotherapy was offered. The patient was started on Sorafenib and initially did well; however, nine days into the treatment, he developed worsening of symptoms. The patient and family declined further treatment due to poor prognosis, and opted for home hospice care. Discussion: This is a unique case of not only an uncommon disease in fibrolamellar carcinoma, but also of a rare presentation with skeletal metastasis. A literature search revealed only one reported case of metastasis to the vertebra in a girl with fibrolamellar carcinoma. However, to the best of our knowledge, no other cases have been reported with bone, lung, lymph node, and retroperitoneal metastasis. Unfortunately, these cases of fibrolamellar carcinoma do not respond to chemotherapy, and prognosis is poor. Patients with unresectable disease have a 5-year median survival of 0-5%, and median survival of ≤12 months.