title: Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Aline Lopes
authors.initials: AL
authors.lastname: Chagas
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Luciana
authors.initials: L
authors.lastname: Kikuchi
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Paulo
authors.initials: P
authors.lastname: Herman
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Regiane S S M
authors.initials: RS
authors.lastname: Alencar
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Claudia M
authors.initials: CM
authors.lastname: Tani
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Márcio Augusto
authors.initials: MA
authors.lastname: Diniz
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Pugliese
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Manoel de Souza
authors.initials: Mde S
authors.lastname: Rocha
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Luiz Augusto Carneiro
authors.initials: LA
authors.lastname: D'Albuquerque
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Flair Jose
authors.initials: FJ
authors.lastname: Carrilho
authors.affiliation: São Paulo Clínicas Liver Cancer Group, São Paulo, Brazil.
authors.forename: Venancio A F
authors.initials: VA
authors.lastname: Alves
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001706:Biopsy; D006528:Carcinoma, Hepatocellular / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology; D009367:Neoplasm Staging; D012307:Risk Factors; D012737:Sex Factors; D015996:Survival Rate; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D047368:Tumor Burden; D055815:Young Adult