Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases
Autor(a) principal: | |
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Data de Publicação: | 2015 |
Outros Autores: | , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Clinics |
Texto Completo: | https://www.revistas.usp.br/clinics/article/view/100957 |
Resumo: | 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. |
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oai:revistas.usp.br:article/100957 |
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Clinics |
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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 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. Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo2015-03-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://www.revistas.usp.br/clinics/article/view/10095710.6061/clinics/2015(03)10Clinics; Vol. 70 No. 3 (2015); 207-213Clinics; v. 70 n. 3 (2015); 207-213Clinics; Vol. 70 Núm. 3 (2015); 207-2131980-53221807-5932reponame:Clinicsinstname:Universidade de São Paulo (USP)instacron:USPenghttps://www.revistas.usp.br/clinics/article/view/100957/99622Copyright (c) 2015 Clinicsinfo:eu-repo/semantics/openAccessChagas, Aline Lopes Kikuchi, Luciana Herman, Paulo Alencar, Regiane S.S.M. Tani, Claudia M. Diniz, Márcio Augusto Pugliese, Vincenzo Rocha, Manoel de Souza D′Albuquerque, Luiz Augusto Carneiro Carrilho, Flair Jose Alves, Venancio A.F. 2015-07-28T13:01:13Zoai:revistas.usp.br:article/100957Revistahttps://www.revistas.usp.br/clinicsPUBhttps://www.revistas.usp.br/clinics/oai||clinics@hc.fm.usp.br1980-53221807-5932opendoar:2015-07-28T13:01:13Clinics - Universidade de São Paulo (USP)false |
dc.title.none.fl_str_mv |
Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases |
title |
Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases |
spellingShingle |
Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases Chagas, Aline Lopes |
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 |
author |
Chagas, Aline Lopes |
author_facet |
Chagas, Aline Lopes Kikuchi, Luciana Herman, Paulo Alencar, Regiane S.S.M. Tani, Claudia M. Diniz, Márcio Augusto Pugliese, Vincenzo Rocha, Manoel de Souza D′Albuquerque, Luiz Augusto Carneiro Carrilho, Flair Jose Alves, Venancio A.F. |
author_role |
author |
author2 |
Kikuchi, Luciana Herman, Paulo Alencar, Regiane S.S.M. Tani, Claudia M. Diniz, Márcio Augusto Pugliese, Vincenzo Rocha, Manoel de Souza D′Albuquerque, Luiz Augusto Carneiro Carrilho, Flair Jose Alves, Venancio A.F. |
author2_role |
author author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Chagas, Aline Lopes Kikuchi, Luciana Herman, Paulo Alencar, Regiane S.S.M. Tani, Claudia M. Diniz, Márcio Augusto Pugliese, Vincenzo Rocha, Manoel de Souza D′Albuquerque, Luiz Augusto Carneiro Carrilho, Flair Jose Alves, Venancio A.F. |
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. |
publishDate |
2015 |
dc.date.none.fl_str_mv |
2015-03-01 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://www.revistas.usp.br/clinics/article/view/100957 10.6061/clinics/2015(03)10 |
url |
https://www.revistas.usp.br/clinics/article/view/100957 |
identifier_str_mv |
10.6061/clinics/2015(03)10 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
https://www.revistas.usp.br/clinics/article/view/100957/99622 |
dc.rights.driver.fl_str_mv |
Copyright (c) 2015 Clinics info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
Copyright (c) 2015 Clinics |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo |
publisher.none.fl_str_mv |
Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo |
dc.source.none.fl_str_mv |
Clinics; Vol. 70 No. 3 (2015); 207-213 Clinics; v. 70 n. 3 (2015); 207-213 Clinics; Vol. 70 Núm. 3 (2015); 207-213 1980-5322 1807-5932 reponame:Clinics instname:Universidade de São Paulo (USP) instacron:USP |
instname_str |
Universidade de São Paulo (USP) |
instacron_str |
USP |
institution |
USP |
reponame_str |
Clinics |
collection |
Clinics |
repository.name.fl_str_mv |
Clinics - Universidade de São Paulo (USP) |
repository.mail.fl_str_mv |
||clinics@hc.fm.usp.br |
_version_ |
1800222761731227648 |