title: Prognostic Value of Relevant Clinicopathologic Variables in Epithelioid Sarcoma: A Multi-Institutional Retrospective Study of 44 Patients.
abstract: BACKGROUND
  Epithelioid sarcoma (ES) is an extremely rare soft tissue sarcoma. Recently, the proximal variant has been reported to be a more aggressive subtype; however, as most reports of ES have involved small case series, the actual prognostic implications remain unclear. We investigated the clinicopathological features of patients with ES to identify the prognostic factors that influence survival.
  
  
  METHODS
  We retrospectively analyzed the clinicopathological features of 44 patients with ES who had been treated at our institutions between 1991 and 2011. Among these patients, 26 were diagnosed histologically as having classic-type ES, whereas the remaining 18 had proximal-type ES. Thirty-three of the patients, all without distant metastases, underwent curative surgery, and the remaining 11 with distant metastases (M1) received palliative treatment.
  
  
  RESULTS
  The proximal subtype was significantly correlated with a proximal tumor location, distant metastases at presentation, presence of rhabdoid cells, a higher tumor grade, and vascular invasion. The overall survival (OS) rate at 5 years for the 44 patients was 45 %. A superficial tumor location and lymph node metastases (N1) at presentation were independently predictive of local recurrence-free survival (LRFS), and N1 and M1 tumors were independently predictive of distant metastasis-free survival and OS, respectively. The proximal subtype was associated with unfavorable LRFS and OS, although not to a statistically significant degree.
  
  
  CONCLUSIONS
  Proximal-type ES has significantly more aggressive clinicopathological features than classic-type ES, and lymph node or distant metastasis has the most critical impact on prognosis.
authors: Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan.
authors: Naofumi
authors: N
authors: Asano
authors: Akihiko
authors: A
authors: Yoshida
authors: Koichi
authors: K
authors: Ogura
authors: Eisuke
authors: E
authors: Kobayashi
authors: Michiro
authors: M
authors: Susa
authors: Hideo
authors: H
authors: Morioka
authors: Shintaro
authors: S
authors: Iwata
authors: Takeshi
authors: T
authors: Ishii
authors: Toru
authors: T
authors: Hiruma
authors: Hirokazu
authors: H
authors: Chuman
authors: Akira
authors: A
authors: Kawai
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D035002:Lower Extremity; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D009361:Neoplasm Invasiveness; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000473:pathology*; D009367:Neoplasm Staging; D018365:Neoplasm, Residual; D010502:Perineum; D012189:Retrospective Studies; D012509:Sarcoma / Q000401:mortality / Q000473:pathology / Q000556:secondary* / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D015996:Survival Rate; D060726:Torso; D034941:Upper Extremity; D055815:Young Adult