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

Abstract

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 T 1 weighted imaging and 54% were hyperintense on T 2 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.

Advances in Fibrolamellar Hepatocellular Carcinoma: A Review

“…4 and 5) that may contain a central scar and/or calcifications. 24,26,27 The value of positron emission tomography (PET) scan for conventional HCC is limited because the sensitivity of PET imaging is not sufficiently high. 28 However, case reports have described unequivocal tracer uptake in FL-HCC, and further study of its role in assisting diagnosis primary or recurrent FL-HCC may be warranted.…”

Imaging features of fibrolamellar hepatocellular carcinoma in gadoxetic acid-enhanced MRI

“…Calcifications are reported to be present in approximately 50% of FLCs and not in FNH but are depicted insufficiently in MRI [ 13 ]. These imaging features are, however, unreliable discriminators: E.g., the signal intensity of the central scar in T2wi has been shown to be variable [ 14 ]. We did not specifically analyze the presence of a central scar and its imaging features in this study, because TACE, which has been performed in 3 of our patients, was considered to affect central scar characterization within treated lesion components.…”

Contemporary management of fibrolamellar hepatocellular carcinoma: diagnosis, treatment, outcome, prognostic factors, and recent developments

“…However, US is nonspecific and less accurate than CT or MRI to differentiate FL-HCC from other mass-forming lesions of the liver. Although CT is adequate for initial pretreatment imaging of FL-HCC, particularly for evaluation of metastatic lesions, MRI may be helpful for initial workup when FL-HCC is first discovered as an initial liver mass [ 22 ]. In general, FL-HCC tends to present as a large, heterogeneous enhancing mass that may contain a central scar and/or calcifications on imaging.…”