title: Surgical Treatment of Paget Disease of the Vulva: Prognostic Significance of Stromal Invasion and Surgical Margin Status.
abstract: OBJECTIVE
  The aim of the study was to evaluate the risk of recurrence according to the surgical margin status and the presence of invasion or of superficially invasive carcinoma in patients with extramammary Paget disease (EMPD) of the vulva, who underwent elective surgical treatment.
  
  
  METHODS
  We performed a retrospective analysis of 27 patients with first diagnosis of extramammary Paget disease of the vulva, who underwent primary and elective surgical treatment from January 1989 to December 2014. A p value of less than .05 was considered statistically significant. Multivariable logistic regression was performed to adjust for confounding factors.
  
  
  RESULTS
  We observed invasive disease in 11 cases, with microinvasion in 8 of them. A positive surgical margin was found in 10 patients. During a median follow-up period of 79.5 months, 8 patients (29.6%) showed a first recurrence after a median (range) time of 4.9 (2.3-7.1) years. No significant differences were observed between patients with recurrence and patients without recurrence with respect to age, number of vulvar sectors involved, bilaterality and multifocality, presence of invasion or microinvasion, and surgical margin status. However, during the follow-up period, the presence of invasion was higher (67% vs 41%) in patients with recurrence compared with patients without recurrence.
  
  
  CONCLUSIONS
  The rate of recurrence of the disease after therapy is high. Patients should be subjected to a close and long-term follow-up to identify those who must undergo further treatment, especially if they presented with an invasive or even microinvasive disease. A free margin of no greater than 1 to 2 cm might be the most appropriate surgical choice.
authors.affiliation: 1Gynecologic Oncology Unit, Centro di Riferimento Oncologico, National Cancer Institute, Aviano; 2Woman's Health Sciences Department, Università Politecnica delle Marche, Ancona; and 3Pathology Unit, Centro di Riferimento Oncologico, National Cancer Institute, Aviano, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Sopracordevole
authors.forename: Jacopo
authors.initials: J
authors.lastname: Di Giuseppe
authors.forename: Giovanni
authors.initials: G
authors.lastname: De Piero
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Canzonieri
authors.forename: Monica
authors.initials: M
authors.lastname: Buttignol
authors.forename: Giorgio
authors.initials: G
authors.lastname: Giorda
authors.forename: Andrea
authors.initials: A
authors.lastname: Ciavattini
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*; D012008:Recurrence; D012189:Retrospective Studies; D018570:Risk Assessment; D014844:Vulva / Q000473:pathology*; D014846:Vulvar Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery*