Fibrolamellar hepatocellular carcinoma: stage at presentation and results of aggressive surgical management.

OBJECTIVE: The purpose of this study was to report the stage of fibrolamellar carcinoma at presentation and the imaging findings of postoperative recurrent tumor in an aggressively managed population and to assess the implications of those findings relative to the patients' management.
MATERIALS AND METHODS: Imaging studies in 10 patients with pathologically proved fibrolamellar carcinoma were reviewed. Preoperative studies included CT (n = 10), sonography (n = 8), and MR imaging (n = 2). Postoperative studies included CT (n = 9), sonography (n = 4), and MR imaging (n = 1). Imaging findings were correlated with clinical and surgical follow-up data. Patients were followed up for 2-75 months (median, 26 months).
RESULTS: At presentation, seven (70%) of 10 patients had metastatic lymphadenopathy. Seven patients (70%), including four with lymph node metastasis, had tumor resections with intent to cure. Postoperative imaging studies revealed recurrent tumor in all seven of these patients, including six patients (86%) who had intrahepatic recurrence with or without lymph node metastasis after 6-18 months, and one patient (14%) who had distant metastases 66 months postoperatively. Recurrent lesions were subsequently resected in three (43%) of seven patients, who were disease-free at a mean of 8 months after their second resection. Five patients died after 9 months mean survival, and two patients were alive with residual tumor after 3 months mean follow-up.
CONCLUSION: Fibrolamellar carcinomas are often of advanced stage at diagnosis. Recurrence after resection with intent to cure is common. Early and frequent follow-up imaging is necessary for optimizing surgical management in patients with fibrolamellar carcinoma.