Hepatobiliary sarcomas are rare neoplasms. They account for less than 1% of malignant hepatobiliary tumours. Due to their protean clinical manifestations, the therapeutic algorithm is not yet standardized. This chapter aims to improve the care of the different types of hepatobiliary sarcomas based on personal experiences, a recent literature review, and data from the European Liver Transplant Registry (ELTR). Partial or total R0 resection should be proposed whenever possible. In the case of multifocal hepatic epithelioid haemangioendothelioma (HEHE), liver transplantation (LT) generates the best long-term disease-free survival (DFS), even when a limited extrahepatic disease is present. Hepatic haemangiosarcoma (HHS) still has an extremely poor prognosis. Partial resection is rarely possible, and LT is an absolute contraindication due to the universal, early tumour recurrence. The same holds for the exceedingly rare hepatic leiomyosarcoma (LMS) and liposarcoma (LPS). Undifferentiated embryonal liver sarcoma (UELS), hepatic rhabdomyosarcoma (RMS), and biliary embryonal rhabdomyosarcoma (BERMS) need combined aggressive (neo-)adjuvant chemotherapy and surgery. In the case of combined sarcomatous hepatocellular cancer (SaHCC), partial or total liver resection results are similar but markedly inferior to those obtained for hepatocellular carcinoma. Definitive inclusion criteria for LT have not yet been identified due to the lack of larger transplant cohorts and also due to the missing long-term follow-up in the large majority of reports. In the absence of extrahepatic disease localization, a radical RO resection should be proposed whenever possible because it always generates the best results. The outlook of hepatobiliary sarcoma patients can be further improved by the identification of prognostic factors as well as the refinement of multimodal treatments combining radical surgery, multidrug or target chemo- and immunotherapy, and external beam radiotherapy. Liver transplantation, especially when implementing living liver donation, will occupy a more important place in this less well-known field of hepatobiliary oncology.

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Hepatobiliary Sarcoma

  • Jan Lerut,
  • Jean de Ville de Goyet,
  • Quirino Lai

摘要

Hepatobiliary sarcomas are rare neoplasms. They account for less than 1% of malignant hepatobiliary tumours. Due to their protean clinical manifestations, the therapeutic algorithm is not yet standardized. This chapter aims to improve the care of the different types of hepatobiliary sarcomas based on personal experiences, a recent literature review, and data from the European Liver Transplant Registry (ELTR). Partial or total R0 resection should be proposed whenever possible. In the case of multifocal hepatic epithelioid haemangioendothelioma (HEHE), liver transplantation (LT) generates the best long-term disease-free survival (DFS), even when a limited extrahepatic disease is present. Hepatic haemangiosarcoma (HHS) still has an extremely poor prognosis. Partial resection is rarely possible, and LT is an absolute contraindication due to the universal, early tumour recurrence. The same holds for the exceedingly rare hepatic leiomyosarcoma (LMS) and liposarcoma (LPS). Undifferentiated embryonal liver sarcoma (UELS), hepatic rhabdomyosarcoma (RMS), and biliary embryonal rhabdomyosarcoma (BERMS) need combined aggressive (neo-)adjuvant chemotherapy and surgery. In the case of combined sarcomatous hepatocellular cancer (SaHCC), partial or total liver resection results are similar but markedly inferior to those obtained for hepatocellular carcinoma. Definitive inclusion criteria for LT have not yet been identified due to the lack of larger transplant cohorts and also due to the missing long-term follow-up in the large majority of reports. In the absence of extrahepatic disease localization, a radical RO resection should be proposed whenever possible because it always generates the best results. The outlook of hepatobiliary sarcoma patients can be further improved by the identification of prognostic factors as well as the refinement of multimodal treatments combining radical surgery, multidrug or target chemo- and immunotherapy, and external beam radiotherapy. Liver transplantation, especially when implementing living liver donation, will occupy a more important place in this less well-known field of hepatobiliary oncology.