title: Role of palliative chemotherapy in advanced epithelioid sarcoma.
abstract: BACKGROUND
  Epithelioid sarcoma is a rare soft tissue sarcoma subtype. The response of this disease to chemotherapy is not well described. The aim of this study was to investigate the response rate and progression-free survival in a series of epithelioid sarcoma patients treated with chemotherapy at a single referral center.
  
  
  METHODS
  A retrospective search of a prospectively maintained database was made to identify epithelioid sarcoma patients treated with chemotherapy between 1990 and 2009. Radiological response and histological diagnosis were re-reviewed for this study.
  
  
  RESULTS
  Twenty-one epithelioid sarcoma patients treated with chemotherapy were identified; follow-up data on palliative chemotherapy was available on 20 of these patients. The median age was 36.5 years (range, 17.4 to 64.8 y) and the male/female ratio was 19:2. Ten patients (50%) were treated with single-agent anthracycline, 9 patients (45%) were treated with a combination therapy (anthracycline and ifosfamide), and 1 patient received trabectedin (5%). Three patients achieved a partial response, 12 had stable disease, and 5 progressed. The median progression-free survival was 29 weeks (95% confidence interval [CI]: 23-35). Seven and 3 patients received second-line and third-line palliative chemotherapy, respectively. The median overall survival from commencing palliative chemotherapy in our series was 51 weeks (95% confidence interval; 29-73).
  
  
  CONCLUSIONS
  Systemic chemotherapy provides satisfactory palliation in patients with epithelioid sarcoma. However, this is an aggressive disease, responses to chemotherapy are of short duration and there is a need for more effective novel therapies in the treatment of this condition.
authors.affiliation: Sarcoma Unit, Royal Marsden Hospital, London, UK. rjones@seattlecca.org
authors.forename: Robin L
authors.initials: RL
authors.lastname: Jones
authors.forename: Anastasia
authors.initials: A
authors.lastname: Constantinidou
authors.forename: David
authors.initials: D
authors.lastname: Olmos
authors.forename: Khin
authors.initials: K
authors.lastname: Thway
authors.forename: Cyril
authors.initials: C
authors.lastname: Fisher
authors.forename: Omar
authors.initials: O
authors.lastname: Al-Muderis
authors.forename: Michelle
authors.initials: M
authors.lastname: Scurr
authors.forename: Ian R
authors.initials: IR
authors.lastname: Judson
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D004149:Dioxoles / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010166:Palliative Care; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D012509:Sarcoma / Q000188:drug therapy* / Q000401:mortality* / Q000473:pathology; D015996:Survival Rate; D044005:Tetrahydroisoquinolines / Q000008:administration & dosage; D000077606:Trabectedin; D055815:Young Adult
chemical_list: D004149:Dioxoles; D044005:Tetrahydroisoquinolines; D004317:Doxorubicin; D000077606:Trabectedin; D007069:Ifosfamide