title: Epithelioid sarcoma: prognostic factors and survival in a series of patients treated at a single institution.
abstract: BACKGROUND
  Epithelioid sarcoma (ES) is a rare subtype of soft-tissue sarcoma of unknown histogenesis. Typically, it occurs superficially as single/multiple nodules (nodular ES), or in deeper tissues as a mass. The correlation between initial presentation and clinical outcome was investigated.
  
  
  METHODS
  Fifty-four consecutive patients surgically treated at a single referral center were retrospectively reviewed. Thirty-six patients presented with a primary and 18 with a recurrent tumor. Potential prognostic clinicopathological variables, including macroscopic features at first presentation, were tested by univariable and multivariable analysis with respect to overall (OS), metastasis-free (MFS), and local recurrence-free survival (LRFS).
  
  
  RESULTS
  The 10-year OS was 61.8% for the whole series. Thirty patients relapsed; in detail, local and distant failure occurred in 14 (25.9%) and 24 (44.4%) patients, respectively. The lymph node involvement rate was 16/54 (29.6%). In both the whole series and the subset of patient with primary ES, single localized tumor correlated with increased OS at multivariable analysis; occurrence of nodal involvement during postoperative follow-up correlated to worse OS and MFS. Nodular ES was an independent predictor of worse LRFS. In univariable analysis, nodular ES was associated with smaller tumor size, distal limb locations, earlier classification of malignant tumor (TNM) stage, and higher amputation rate. A statistical difference in the pattern of failure between nodular and mass ES was found.
  
  
  CONCLUSIONS
  Primary tumor macroscopic features seem to correlate to different local aggressiveness and failure patterns. Better prognosis is associated with single localized disease stage and no occurrence of locoregional spread.
authors.affiliation: Department of Surgery, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
authors.forename: Dario
authors.initials: D
authors.lastname: Baratti
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Pennacchioli
authors.forename: Paolo G
authors.initials: PG
authors.lastname: Casali
authors.forename: Rossella
authors.initials: R
authors.lastname: Bertulli
authors.forename: Laura
authors.initials: L
authors.lastname: Lozza
authors.forename: Patrizia
authors.initials: P
authors.lastname: Olmi
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Stefano
authors.initials: S
authors.lastname: Radaelli
authors.forename: Marco
authors.initials: M
authors.lastname: Fiore
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gronchi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D015622:Epithelioid Cells / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D011379:Prognosis; D012189:Retrospective Studies; D012509:Sarcoma / Q000401:mortality* / Q000473:pathology; D012983:Soft Tissue Neoplasms / Q000401:mortality* / Q000473:pathology; D015996:Survival Rate