title: Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis.
abstract: BACKGROUND
  Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited. The main objective of this study was to investigate the activity of pazopanib in vascular sarcomas.
  
  
  METHODS
  A retrospective study of patients with advanced vascular sarcomas, including angiosarcoma (AS), epithelioid hemangioendothelioma (HE) and intimal sarcoma (IS) treated with pazopanib in real life practice at EORTC centers as well as patients treated within the EORTC phase II and III clinical trials (62043/62072) was performed. Patient and tumor characteristics were collected. Response was assessed according to RECIST 1.1. and survival analysis was performed.
  
  
  RESULTS
  Fifty-two patients were identified, 40 (76.9%), 10 (19.2%) and two (3.8%) with AS, HE and IS, respectively. The response rate was eight (20%), two (20%) and two (100%) in the AS, HE and IS subtypes, respectively. There was no significant difference in response rate between cutaneous and non-cutaneous AS and similarly between radiation-associated and non-radiation-associated AS. Median progression-free survival (PFS) and median overall survival (OS; from commencing pazopanib) were three months (95% CI 2.1-4.4) and 9.9 months (95% CI 6.5-11.3) in AS, respectively.
  
  
  CONCLUSIONS
  The activity of pazopanib in AS is comparable to its reported activity in other STS subtypes. In this study, the activity of pazopanib was similar in cutaneous/non-cutaneous and in radiation/non-radiation-associated AS. In addition, pazopanib showed promising activity in HE and IS, worthy of further evaluation.
authors.affiliation: a Sarcoma Unit, Department of Medical Oncology , University Hospital of Bern , Bern , Switzerland.
authors.forename: A
authors.initials: A
authors.lastname: Kollár
authors.affiliation: b Sarcoma Unit , Royal Marsden NHS Foundation Trust and Institute of Cancer Research , London , UK.
authors.forename: R L
authors.initials: RL
authors.lastname: Jones
authors.affiliation: c Sarcoma Unit, Department of Cancer Medicine , Fondazione IRCCS Istituto Nazionale Tumori , Milan , Italy.
authors.forename: S
authors.initials: S
authors.lastname: Stacchiotti
authors.affiliation: d Department of Medical Oncology , Leiden University Medical Center , RC Leiden , The Netherlands.
authors.forename: H
authors.initials: H
authors.lastname: Gelderblom
authors.affiliation: e Oncology Department , National Cancer Institute "Giovanni Paolo II" , Bari , Italy.
authors.forename: M
authors.initials: M
authors.lastname: Guida
authors.affiliation: f Division of Medical Oncology , Candiolo Cancer Institue-FPO, IRCCS , Candiolo , Italy.
authors.forename: G
authors.initials: G
authors.lastname: Grignani
authors.affiliation: g Department of Medical Oncology , Pharmacology the Netherlands Cancer Institute , Amsterdam , The Netherlands.
authors.forename: N
authors.initials: N
authors.lastname: Steeghs
authors.affiliation: h Department of Oncology , Aarhus University Hospital , Aarhus , Denmark.
authors.forename: A
authors.initials: A
authors.lastname: Safwat
authors.affiliation: i Sharett Institute of Oncology , Hadassah-Hebrew University Medical Center, Kiryat Hadassah , Jerusalem , Israel.
authors.forename: D
authors.initials: D
authors.lastname: Katz
authors.affiliation: j La Timone University Hospital & Aix-Marseille University (AMU) , Marseille , France.
authors.forename: F
authors.initials: F
authors.lastname: Duffaud
authors.affiliation: k Department of Medical Oncology , Erasmus MC-Cancer Institute, Erasmus University Medical Center , CE Rotterdam , The Netherlands.
authors.forename: S
authors.initials: S
authors.lastname: Sleijfer
authors.affiliation: b Sarcoma Unit , Royal Marsden NHS Foundation Trust and Institute of Cancer Research , London , UK.|l Department of Medical Oncology , Radboud University Medical Center , GA Nijmegen , The Netherlands.
authors.forename: W T
authors.initials: WT
authors.lastname: van der Graaf
authors.affiliation: m European Organization for Research and Treatment of Cancer (EORTC) , Bruxelles , Belgium.
authors.forename: N
authors.initials: N
authors.lastname: Touati
authors.affiliation: m European Organization for Research and Treatment of Cancer (EORTC) , Bruxelles , Belgium.
authors.forename: S
authors.initials: S
authors.lastname: Litière
authors.affiliation: m European Organization for Research and Treatment of Cancer (EORTC) , Bruxelles , Belgium.
authors.forename: S
authors.initials: S
authors.lastname: Marreaud
authors.affiliation: n Department of Surgery , Fondazione IRCCS Istituto Nazionale dei Tumori , Milano , Italy.
authors.forename: A
authors.initials: A
authors.lastname: Gronchi
authors.affiliation: o Sarcoma Unit, Interdisciplinary Tumor Center , Mannheim University Medical Center, University of Heidelberg , Mannheim , Germany.
authors.forename: B
authors.initials: B
authors.lastname: Kasper
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use*; D005260:Female; D005500:Follow-Up Studies; D018323:Hemangioendothelioma, Epithelioid / Q000098:blood supply / Q000188:drug therapy* / Q000556:secondary; D006394:Hemangiosarcoma / Q000098:blood supply / Q000188:drug therapy* / Q000556:secondary; D006801:Humans; D007191:Indazoles; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D009369:Neoplasms / Q000188:drug therapy* / Q000473:pathology; D011379:Prognosis; D011743:Pyrimidines / Q000627:therapeutic use*; D012189:Retrospective Studies; D013449:Sulfonamides / Q000627:therapeutic use*; D015996:Survival Rate; D017539:Tunica Intima / Q000187:drug effects* / Q000473:pathology
chemical_list: D020533:Angiogenesis Inhibitors; D007191:Indazoles; D011743:Pyrimidines; D013449:Sulfonamides; C516667:pazopanib