Fibrolamellar hepatocellular carcinoma (FL-HCC) is an aggressive and rare variant of hepatocellular carcinoma (HCC) that primarily affects young adults in the absence of pre-existing liver disease or cirrhosis. It has a high recurrence following surgical resection. Patients usually present with abdominal pain and hepatomegaly. Paraneoplastic manifestations in FL-HCC are very rare compared with HCC, however gynecomastia, hypoglycaemia and hyperthyroidism have been previously reported. To date, there are no cases or studies demonstrating an association between FL-HCC and neuropsychiatric manifestations and the curative effect of surgery.
The presence of neuropsychiatric paraneoplastic manifestations in FL-HCC are an unprecedented phenomena. The diagnosis is made in the presence or absence of serum anti-neuronal antibodies, normal imaging and neurological studies and the exclusion of alternative neurological and psychiatric conditions.
Case presentation: A 19 year old female with a background of paranoid thought disorder was incidentally found to have a large (10 cm) liver lesion on CT lumbar spine for the investigation of left lower limb weakness. A triple phase CT of the liver was suggestive of a 10x6.0x7.0 cm FL-HCC incorporating segments II, II, IV, V and VIII. Alpha-feto protein levels and liver function tests were normal. She underwent an extended left hepatectomy with complete resolution of lower limb neurological symptoms and a reduction of paranoid thoughts following curative resection. Histopathology confirmed a FL-HCC (T2N1). Pre-operative assessment including negative serum anti-neuronal antibodies, MRI brain and whole spine were not suggestive of metastasis. They did not identify a central or peripheral neurological pathology. The rapid improvement of symptoms post-operatively precluded the requirement for electromyography. There were no alternative explanations to account for the observed phenomena.
The purpose of this presentation is to demonstrate the rare association between FL-HCC and neuropsychiatric paraneoplastic manifestations and the curative effect of surgery. Further research is required to investigate this observed association.