title: Epithelioid sarcoma: one institution's experience with a rare sarcoma.
abstract: BACKGROUND
  Epithelioid sarcomas (ES) are extremely rare soft tissue sarcomas. As such, their clinical behavior and response to treatment are poorly described in the literature.
  
  
  METHODS
  We queried the centralized cancer registry and pathology archives at the Johns Hopkins Medical Institution and identified 22 patients with a diagnosis of ES. We excluded two patients because of inadequate data. A pathologist reviewed patient charts and reexamined available histological slides. This study was performed with institutional review board approval.
  
  
  RESULTS
  The median age at diagnosis was 27.8 y; most patients (75%) were male. Regional lymph node metastases were present in 10% of patients at presentation. The majority of tumors (57.9%) recurred and 35% recurred more than once, although the number of recurrences did not affect survival (P = 0.48). Patients did not experience a decrease in time to recurrence with increasing number of resections. The median time between resection and recurrence was 1.23 y and the maximum was 18.8 y. Median overall survival was 56.2 mo and 5-y survival was 92%.
  
  
  CONCLUSIONS
  Our study reveals that ES is an extremely rare tumor with a protracted and recurrent course, but overall survival may be more favorable than in the past. Patients benefit from aggressive and repeated resection. Epithelioid sarcoma is unique because it metastasizes to regional nodal basins. Extended surveillance is indicated, because recurrences can appear after decades of quiescence.
authors.affiliation: Department of Surgery, Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
authors.forename: Angela A
authors.initials: AA
authors.lastname: Guzzetta
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Montgomery
authors.forename: Heather
authors.initials: H
authors.lastname: Lyu
authors.forename: Craig M
authors.initials: CM
authors.lastname: Hooker
authors.forename: Christian F
authors.initials: CF
authors.lastname: Meyer
authors.forename: David M
authors.initials: DM
authors.lastname: Loeb
authors.forename: Deborah
authors.initials: D
authors.lastname: Frassica
authors.forename: Kristy L
authors.initials: KL
authors.lastname: Weber
authors.forename: Nita
authors.initials: N
authors.lastname: Ahuja
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008396:Maryland / Q000453:epidemiology; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology*; D012509:Sarcoma / Q000453:epidemiology* / Q000628:therapy; D055815:Young Adult