title: Biology and management of clear cell sarcoma: state of the art and future perspectives.
abstract: BACKGROUND
  Clear cell sarcoma (CCS) is an aggressive tumor, typically developing in tendons or aponeuroses. The outcome of this orphan disease is poor, with 5-year and 10-year survival rates of localized CCS around 60-70% and 40-50%. Once the disease has metastasized, it is usually fatal due to its chemotherapy-resistant nature. Systemic treatment options are poorly standardized and the use of chemotherapy is based on weak scientific evidence.
  
  
  METHODS
  In this review, we systematically discuss the current scientific evidence for the systemic treatment of CCS, including tyrosine kinase inhibitors, immunotherapy and MET inhibitors. Expert commentary: Recent insights in the biology of CCS have identified new potential therapeutic targets, which should be tested in prospective clinical trials. Whenever possible, patients with metastatic CCS should be included in clinical trials with good biological rationale. Innovative trial methodology and new regulatory mechanisms are required to provide patients with uncommon cancers with active drugs.
authors.affiliation: a Laboratory of Experimental Oncology, Department of Oncology, KU Leuven and Department of General Medical Oncology , University Hospitals Leuven, Leuven Cancer Institute , Leuven , Belgium.
authors.forename: Jasmien
authors.initials: J
authors.lastname: Cornillie
authors.affiliation: a Laboratory of Experimental Oncology, Department of Oncology, KU Leuven and Department of General Medical Oncology , University Hospitals Leuven, Leuven Cancer Institute , Leuven , Belgium.
authors.forename: Thomas
authors.initials: T
authors.lastname: van Cann
authors.affiliation: a Laboratory of Experimental Oncology, Department of Oncology, KU Leuven and Department of General Medical Oncology , University Hospitals Leuven, Leuven Cancer Institute , Leuven , Belgium.
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Wozniak
authors.affiliation: b Department of Surgical Oncology , KU Leuven and University Hospitals Leuven , Leuven , Belgium.
authors.forename: Daphne
authors.initials: D
authors.lastname: Hompes
authors.affiliation: a Laboratory of Experimental Oncology, Department of Oncology, KU Leuven and Department of General Medical Oncology , University Hospitals Leuven, Leuven Cancer Institute , Leuven , Belgium.
authors.forename: Patrick
authors.initials: P
authors.lastname: Schöffski
mesh_terms: D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D019008:Drug Resistance, Neoplasm; D006801:Humans; D007167:Immunotherapy / Q000379:methods*; D009362:Neoplasm Metastasis; D047428:Protein Kinase Inhibitors / Q000494:pharmacology / Q000627:therapeutic use; D019859:Proto-Oncogene Proteins c-met / Q000037:antagonists & inhibitors; D018227:Sarcoma, Clear Cell / Q000473:pathology / Q000628:therapy*; D015996:Survival Rate
chemical_list: D000970:Antineoplastic Agents; D047428:Protein Kinase Inhibitors; D019859:Proto-Oncogene Proteins c-met
keywords: Clear cell sarcoma; EWSR1-ATF1 fusion protein; MET; chemotherapy resistance; immunotherapy; malignant melanoma of soft parts; molecular targets