Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases

  • Aline Lopes Chagas Universidade de São Paulo; Faculdade de Medicina; Instituto do Câncer do Estado de São Paulo; Universidade de São Paulo
  • Luciana Kikuchi Universidade de São Paulo; Faculdade de Medicina; Instituto do Câncer do Estado de São Paulo; Universidade de São Paulo
  • Paulo Herman Universidade de São Paulo; Faculdade de Medicina; Department of Gastroenterology; Hospital das Clínicas; Universidade de São Paulo
  • Regiane S.S.M. Alencar Universidade de São Paulo; Faculdade de Medicina; Instituto do Câncer do Estado de São Paulo; Universidade de São Paulo
  • Claudia M. Tani Universidade de São Paulo; Faculdade de Medicina; Instituto do Câncer do Estado de São Paulo; Universidade de São Paulo
  • Márcio Augusto Diniz Universidade de São Paulo; Faculdade de Medicina; Department of Gastroenterology; Hospital das Clínicas; Universidade de São Paulo
  • Vincenzo Pugliese Universidade de São Paulo; Faculdade de Medicina; Department of Gastroenterology; Hospital das Clínicas; Universidade de São Paulo
  • Manoel de Souza Rocha Universidade de São Paulo; Faculdade de Medicina; Department of Radiology; Hospital das Clínicas; Universidade de São Paulo
  • Luiz Augusto Carneiro D′Albuquerque Universidade de São Paulo; Faculdade de Medicina; Department of Gastroenterology; Hospital das Clínicas; Universidade de São Paulo
  • Flair Jose Carrilho Universidade de São Paulo; Faculdade de Medicina; Department of Gastroenterology; Hospital das Clínicas; Universidade de São Paulo
  • Venancio A.F. Alves Universidade de São Paulo; Faculdade de Medicina; Department of Pathology; Hospital das Clínicas; Universidade de São Paulo

Abstract

OBJECTIVES: Fibrolamellar hepatocellular carcinoma is a rare primary malignant liver tumor that differs from conventional hepatocellular carcinoma in several aspects. The aim of this study was to describe the clinical, surgical and histopathological features of fibrolamellar hepatocellular carcinoma and to analyze the factors associated with survival. METHODS: We identified 21 patients with histopathologically diagnosed fibrolamellar hepatocellular carcinoma over a 22-year period. Clinical information was collected from medical records and biopsies, and surgical specimens were reviewed. RESULTS: The median age at diagnosis was 20 years. Most patients were female (67%) and did not have associated chronic liver disease. Most patients had a single nodule, and the median tumor size was 120 mm. Vascular invasion was present in 31% of patients, and extra-hepatic metastases were present in 53%. Fourteen patients underwent surgery as the first-line therapy, three received chemotherapy, and four received palliative care. Eighteen patients had “pure fibrolamellar hepatocellular carcinoma,” whereas three had a distinct area of conventional hepatocellular carcinoma and were classified as having “mixed fibrolamellar hepatocellular carcinoma.” The median overall survival was 36 months. The presence of “mixed fibrolamellar hepatocellular carcinoma” and macrovascular invasion were predictors of poor survival. Vascular invasion was associated with an increased risk of recurrence in patients who underwent surgery. CONCLUSION: Fibrolamellar hepatocellular carcinoma was more common in young female patients without chronic liver disease. Surgery was the first therapeutic option to achieve disease control, even in advanced cases. Vascular invasion was a risk factor for tumor recurrence. The presence of macrovascular invasion and areas of conventional hepatocellular carcinoma were directly related to poor survival.

Downloads

Download data is not yet available.
Published
2015-03-01
How to Cite
Chagas, A., Kikuchi, L., Herman, P., Alencar, R., Tani, C., Diniz, M., Pugliese, V., Rocha, M., D′Albuquerque, L., Carrilho, F., & Alves, V. (2015). Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases . Clinics, 70(3), 207-213. https://doi.org/10.6061/clinics/2015(03)10
Section
Clinical Sciences