title: Prognostic factors in alveolar soft part sarcoma: A SEER analysis.
abstract: OBJECTIVE
  We reviewed the clinical characteristics and outcomes of patients treated for alveolar soft part sarcoma (ASPS) and analyzed the effect of surgery for patients presenting with and without metastatic disease (DM).
  
  
  METHODS
  The SEER Registry was queried for patients with ASPS from 1973-2012. The Kaplan-Meier estimate and Cox proportional hazards were used to analyze survival outcomes and risk variables.
  
  
  RESULTS
  Among 251 patients, 43% had DM and 67% locoregional disease (LR) on presentation. The 5-year overall survival (OS) for all patients was 56% (82% and 27% for LR and DM, respectively). Multivariate analysis identified older age (hazard ratio [HR] = 1.03 per year, P < 0.001), tumor size >10 cm (HR = 2.76, P = 0.013), DM at diagnosis (HR = 3.79, P < 0.001), and truncal primary site (HR = 1.63, P = 0.035) as independent factors predicting worse OS. For LR patients, surgery plus radiotherapy (RT) resulted in better OS compared to surgery alone P = 0.014. For DM patients, primary site surgery significantly improved survival (P < 0.001).
  
  
  CONCLUSIONS
  ASPS presents with high metastasis rate but has a relatively indolent clinical course and a favorable prognosis with prolonged survival. Aggressive treatment using adjuvant RT with surgery is indicated in patients with LR disease and surgery is indicated in patients presenting with DM. J. Surg. Oncol. 2016;113:581-586. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Haotong
authors.initials: H
authors.lastname: Wang
authors.affiliation: Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Alex
authors.initials: A
authors.lastname: Jacobson
authors.affiliation: Department of Medical Oncology, Massachusetts General Hospital, Boston, Massachusetts.|Harvard Medical School, Boston, Massachusetts.
authors.forename: David C
authors.initials: DC
authors.lastname: Harmon
authors.affiliation: Department of Medical Oncology, Massachusetts General Hospital, Boston, Massachusetts.|Harvard Medical School, Boston, Massachusetts.
authors.forename: Edwin
authors.initials: E
authors.lastname: Choy
authors.affiliation: Harvard Medical School, Boston, Massachusetts.|Department of Orthopedic Oncology, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Francis J
authors.initials: FJ
authors.lastname: Hornicek
authors.affiliation: Harvard Medical School, Boston, Massachusetts.|Department of Orthopedic Oncology, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Kevin A
authors.initials: KA
authors.lastname: Raskin
authors.affiliation: Harvard Medical School, Boston, Massachusetts.|Department of Pathology, Massachusetts General Hospital, Boston, Massachusetts.
authors.forename: Ivan A
authors.initials: IA
authors.lastname: Chebib
authors.affiliation: Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts.|Harvard Medical School, Boston, Massachusetts.
authors.forename: Thomas F
authors.initials: TF
authors.lastname: DeLaney
authors.affiliation: Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts.|Harvard Medical School, Boston, Massachusetts.
authors.forename: Yen-Lin E
authors.initials: YL
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018426:SEER Program; D018234:Sarcoma, Alveolar Soft Part / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D015996:Survival Rate; D014481:United States / Q000453:epidemiology; D055815:Young Adult
keywords: ASPS; soft tissue sarcoma; surgical treatment; survival