Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases
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...
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2015-03-01
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doaj-e75220a69e4e449b9c73f9ea019d592d2020-11-24T23:24:10ZengFaculdade de Medicina / USPClinics1980-53222015-03-0170320721310.6061/clinics/2015(03)10S1807-59322015000300207Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 casesAline Lopes ChagasLuciana KikuchiPaulo HermanRegiane S.S.M. AlencarClaudia M. TaniMárcio Augusto DinizVincenzo PuglieseManoel de Souza RochaLuiz Augusto Carneiro D′AlbuquerqueFlair Jose CarrilhoVenancio A.F. AlvesOBJECTIVES: 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322015000300207&lng=en&tlng=enFibrolamellar hepatocellular carcinomaLiver cancerLiver neoplasmsHepatocellular carcinoma (fibrolamellar variant) |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Aline Lopes Chagas Luciana Kikuchi Paulo Herman Regiane S.S.M. Alencar Claudia M. Tani Márcio Augusto Diniz Vincenzo Pugliese Manoel de Souza Rocha Luiz Augusto Carneiro D′Albuquerque Flair Jose Carrilho Venancio A.F. Alves |
spellingShingle |
Aline Lopes Chagas Luciana Kikuchi Paulo Herman Regiane S.S.M. Alencar Claudia M. Tani Márcio Augusto Diniz Vincenzo Pugliese Manoel de Souza Rocha Luiz Augusto Carneiro D′Albuquerque Flair Jose Carrilho Venancio A.F. Alves Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases Clinics Fibrolamellar hepatocellular carcinoma Liver cancer Liver neoplasms Hepatocellular carcinoma (fibrolamellar variant) |
author_facet |
Aline Lopes Chagas Luciana Kikuchi Paulo Herman Regiane S.S.M. Alencar Claudia M. Tani Márcio Augusto Diniz Vincenzo Pugliese Manoel de Souza Rocha Luiz Augusto Carneiro D′Albuquerque Flair Jose Carrilho Venancio A.F. Alves |
author_sort |
Aline Lopes Chagas |
title |
Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases |
title_short |
Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases |
title_full |
Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases |
title_fullStr |
Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases |
title_full_unstemmed |
Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases |
title_sort |
clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases |
publisher |
Faculdade de Medicina / USP |
series |
Clinics |
issn |
1980-5322 |
publishDate |
2015-03-01 |
description |
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. |
topic |
Fibrolamellar hepatocellular carcinoma Liver cancer Liver neoplasms Hepatocellular carcinoma (fibrolamellar variant) |
url |
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322015000300207&lng=en&tlng=en |
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