OBJECTIVES: @entity11469 is a rare primary @entity96 that differs from @entity157 in several aspects. The aim of this study was to describe the clinical, surgical and histopathological features of @entity11469 and to analyze the factors associated with survival. METHODS: We identified 21 @entity1 with histopathologically diagnosed @entity11469 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 @entity1 were female (67%) and did not have associated @entity409 . Most @entity1 had a single nodule, and the median @entity5 size was 120 mm. Vascular invasion was present in 31% of @entity1 , and extra-hepatic metastases were present in 53%. Fourteen @entity1 underwent surgery as the first-line therapy, three received chemotherapy, and four received palliative care. Eighteen @entity1 had "pure @entity11469 ," whereas three had a distinct area of @entity157 and were classified as having "mixed @entity11469 ." The median overall survival was 36 months. The presence of "mixed @entity11469 " and macrovascular invasion were predictors of poor survival. Vascular invasion was associated with an increased risk of recurrence in @entity1 who underwent surgery. CONCLUSION: @entity11469 was more common in young female @entity1 without @entity409 . Surgery was the first therapeutic option to achieve disease control, even in advanced cases. Vascular invasion was a risk factor for @entity5 recurrence. The presence of macrovascular invasion and areas of @entity157 were directly related to poor survival.

Clinical and pathological evaluation of XXXX : a single center study of 21 cases.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity96 :: ('MESH:D008113', 'Disease') :: ['malignant liver tumor'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor'] @entity11469 :: ('MESH:C537258', 'Disease') :: ['Fibrolamellar hepatocellular carcinoma', 'fibrolamellar hepatocellular carcinoma'] @entity409 :: ('MESH:D008107', 'Disease') :: ['chronic liver disease'] @entity157 :: ('MESH:D006528', 'Disease') :: ['conventional hepatocellular carcinoma']
@entity11469 :: (MESH:C537258,Disease) :: ['fibrolamellar hepatocellular carcinoma']
