Sarcoma primário de fígado: revisão sistemática

Abstract

Introduction/Objective: Primary liver sarcomas are a rare type of tumor witch is more common in children. In the adult population, it can be presented as a spectrum of various neoplasms with a reserved prognosis. Besides, there is no consensus of the best therapeutic to be used in the treatment of those lesions. Therefore, this Systematic Review was made to find the best treatment options (surgery and/or chemotherapy and/or radiotherapy), to determinate prognostic factors and overall survival (OS). Material and Methods: Systematic review of articles published on PUBMED and MEDLINE (1966 to march 2016), with keywords: Primary liver sarcoma and Primary hepatic sarcoma. Adult patients (>18 years), articles in English, Portuguese and Spanish were included. Case reports, tumors of double origin (carcinosarcomas), and patients with metastasis and multiple diagnosis were excluded. Three authors researched individually and the senior author judged the inclusion of the pre-selected articles. After the research, 1314 articles were found, of those 1206 were excluded because they were clearly out of inclusion criteria. In the end were left 108 articles and, after reading all the abstracts, 12 were included. Results: The most common histologic types were leiomyosarcoma, angiosarcoma and epithelioid hemangioendothelioma. Histologic type, differentiation degree and R0 resection were the most influent prognostic factors. The 5 year OS varied from 18% to 65%. Conclusion: Surgery (resection) is the main treatment of primary liver sarcomas. More effective systemic treatments need to be developed to improve the prognosis of this heterogeneous type of tumor.