CT and MRI of primary and metastatic fibrolamellar carcinoma: a case series of 37 patients

OBJECTIVE: Fibrolamellar carcinoma (FLC) is a rare disease, with limited radiographic reported information. We assessed the imaging patterns of primary and metastatic FLC. METHODS: CT and MR examinations of patients with FLC were retrospectively reviewed. Imaging features were assessed for primary and recurrent liver tumours, including dimension, enhancement characteristics, and presence or absence of central scars. Locations of nodal and extranodal metastases were also recorded. RESULTS: Of 37 patients (18 males and 19 females; average age, 23.5 years) with FLC, 24 had imaging of their primary tumour; 13 had metastases at presentation and 7 developed metastases on follow-up. The remaining 13 patients had follow-up imaging of metastatic disease. Primary FLC had a mean diameter >11 cm, with central scars in ten (46%) patients. Most tumours enhanced heterogeneously (96%) and showed arterial enhancement (81%). On MRI, 62% of FLCs were hypointense on T1 weighted imaging and 54% were hyperintense on T2 weighted imaging. 13 patients (54%) had nodal metastases at presentation, mostly in the upper abdomen (92%) and commonly in the chest (38%). Extrahepatic metastases were most frequently pulmonary or peritoneal. Predominantly small and homogeneous intrahepatic recurrences were detected on follow-up in 15 patients. CONCLUSION: FLC often presents as a large hepatic tumour with nodal and distant metastases. Thoracic adenopathy and lung metastases were frequently found in our series, suggesting the need for pre-operative and follow-up chest imaging. ADVANCES IN KNOWLEDGE: Thoracic nodal and lung metastases are common in FLC; therefore, dedicated chest imaging should be part of the evaluation of a patient with FLC.