title: [Clinical characterization of vulvar epithelioid sarcoma].
abstract: OBJECTIVE
  Vulvar epithelioid sarcoma is a rare, undifferentiated soft-tissue sarcoma, with a high rate of local relapse, regional nodal spread and distant metastases. The aim of this study was to investigate the clinical features, diagnosis, treatment and prognosis of this malignancy.
  
  
  METHODS
  We studied the clinicopathologic features of 20 cases of vulvar epithelioid sarcoma, of which 4 cases were admitted to our hospital from 1999 to 2009. All of the patients received radical local excision with inguinofemoral lymphadenectomy. Seven patients were treated without adjuvant therapy. Seven patients received postoperative radiotherapy only and three underwent chemotherapy. Chemotherapy plus radiotherapy were given postoperatively in three.
  
  
  RESULTS
  The patients ranged in age from 23 to 80 years (median: 36 y). The tumors ranged from 1 to 10 cm in their greatest diameter (median: 5.1 cm). All cases showed immunoreactivity for both vimentin and cytokeratin. Follow-up information on all 20 patients was available, and covered periods ranging from 3 to 104 months.11 patients were alive with no evidence of disease. 2 patients developed lymph node metastases but alive. 7 patients had died of the disease. Survival of the early stage (I-II) patients was significantly longer than those in the advanced stage (III-IV) (median, 21 vs. 6 months, P < 0.01). There was no significant difference between survival of patients with or without inguinofemoral lymphadenectomy (median, 11.5 vs. 6 months, P = 0.086).
  
  
  CONCLUSIONS
  Because of the relatively frequent misdiagnosis, a differential diagnosis combined with immunohistochemistry is needed to determine an early and accurate diagnosis. The tumor markers exhibiting immunoreactivity includ vimentin, epithelial membrane antigen (EMA) and cytokeratin (CK). Radical local excision with adequate margin (at least 2 cm) and bilateral inguinofemoral lymphadenectomy is effective for the treatment of vulvar epithelioid sarcoma. The role of adjuvant therapy, chemotherapy and radiation remains unclear but merits consideration.
authors.affiliation: Department of Gynecological Oncology, Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China.
authors.forename: Li
authors.initials: L
authors.lastname: Sun
authors.forename: Ling-Ying
authors.initials: LY
authors.lastname: Wu
authors.forename: Xiao-Guang
authors.initials: XG
authors.lastname: Li
authors.forename: Ping
authors.initials: P
authors.lastname: Bai
authors.forename: Hong-Tu
authors.initials: HT
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017024:Chemotherapy, Adjuvant; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007633:Keratins / Q000378:metabolism; D008197:Lymph Node Excision; D008207:Lymphatic Metastasis; D008875:Middle Aged; D018396:Mucin-1 / Q000378:metabolism; D009367:Neoplasm Staging; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012509:Sarcoma / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery*; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery*; D015996:Survival Rate; D014746:Vimentin / Q000378:metabolism; D014844:Vulva / Q000601:surgery; D014846:Vulvar Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology* / Q000532:radiotherapy / Q000601:surgery*; D055815:Young Adult
chemical_list: D018396:Mucin-1; D014746:Vimentin; D007633:Keratins