Publicação
Managing Advanced Fibrolamellar Carcinoma at Stage IV: A Case Report.
| Resumo: | Introduction: Fibrolamellar carcinoma (FLC) is a rare primary liver malignancy that predominantly affects young individuals without underlying liver disease, distinguishing it from typical hepatocellular carcinoma. It is characterized by the DNAJB1-PRKACA fusion gene, which is believed to drive tumorigenesis. The diagnosis is often delayed due to nonspecific symptoms. Disease stage and resectability are the most significant prognostic factors. Surgery remains the only potentially curative option, but many patients present with unresectable or metastatic disease, for which there is no established standard treatment. For unresectable and metastatic disease, treatment decisions are largely empirical and based on case series. Case presentation: We report a case of a 29-year-old woman diagnosed with metastatic FLC who was treated with 6 cycles of gemcitabine + oxaliplatin, achieving stable disease for nearly 1 year. However, she subsequently experienced disease progression and rapidly declined, preventing the initiation of further systemic therapy. Discussion/conclusion: This case highlights the limitations of current systemic therapies and the urgent need for novel targeted treatments. Further research is essential to improve outcomes for patients with metastatic FLC. |
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| Autores principais: | Furtado, Ivánia |
| Outros Autores: | Gião, Nuno; Urbano, Martim; Ferreira, João Boavida |
| Assunto: | Fibrolamellar carcinoma Gemcitabine Hepatocellular carcinoma Neoplasm metastasis Oxaliplatin HSAC ONC HSAC GAS HCC PAT CLIN |
| Ano: | 2026 |
| País: | Portugal |
| Tipo de documento: | texto |
| Tipo de acesso: | acesso aberto |
| Instituição associada: | Centro Hospitalar de Lisboa Central, EPE (CHLC) |
| Idioma: | inglês |
| Origem: | Repositório do Centro Hospitalar de Lisboa Central, EPE |
| Resumo: | Introduction: Fibrolamellar carcinoma (FLC) is a rare primary liver malignancy that predominantly affects young individuals without underlying liver disease, distinguishing it from typical hepatocellular carcinoma. It is characterized by the DNAJB1-PRKACA fusion gene, which is believed to drive tumorigenesis. The diagnosis is often delayed due to nonspecific symptoms. Disease stage and resectability are the most significant prognostic factors. Surgery remains the only potentially curative option, but many patients present with unresectable or metastatic disease, for which there is no established standard treatment. For unresectable and metastatic disease, treatment decisions are largely empirical and based on case series. Case presentation: We report a case of a 29-year-old woman diagnosed with metastatic FLC who was treated with 6 cycles of gemcitabine + oxaliplatin, achieving stable disease for nearly 1 year. However, she subsequently experienced disease progression and rapidly declined, preventing the initiation of further systemic therapy. Discussion/conclusion: This case highlights the limitations of current systemic therapies and the urgent need for novel targeted treatments. Further research is essential to improve outcomes for patients with metastatic FLC. |
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