title: Extramammary Paget's disease: treatment, prognostic factors and outcome in 76 patients.
abstract: BACKGROUND
  Extramammary Paget's disease (EMPD) is a rare cutaneous carcinoma usually presenting as a genital erythematous lesion in the elderly. Although most EMPD tumours are in situ, invasive EMPD has a poor prognosis.
  
  
  OBJECTIVE
  To evaluate the clinical and pathological features of EMPD and determine prognostic factors for survival.
  
  
  METHODS
  The medical records of 76 patients with EMPD were retrospectively reviewed.
  
  
  RESULTS
  Of the 66 patients who underwent curative surgical excision, five (8%) developed local recurrence, but surgical margin (<or=2 cm or >2 cm) was not correlated with local recurrence. Thirteen of the 76 patients (17%) developed systemic metastases and 10 of these died of disease. On univariate analysis, the presence of nodules in the primary tumour, clinical lymph node swelling, elevated serum carcinoembryonic antigen (CEA) levels, tumour invasion level and lymph node metastasis were significant prognostic factors. On multivariate analysis, invasion level and elevated serum CEA were the only factors that were significantly associated with reduced survival.
  
  
  CONCLUSIONS
  Invasion level and lymph node metastasis are important prognostic factors in EMPD. In patients with in situ tumour, local tumour control is the major aim of treatment; however, wide surgical margins are not associated with a lower risk of local recurrence.
authors.affiliation: Division of Dermatology, Toyama Prefectural Central Hospital, Nishinagae, Toyama 930-8550, Japan. hattanao@tch.pref.toyama.jp
authors.forename: N
authors.initials: N
authors.lastname: Hatta
authors.forename: M
authors.initials: M
authors.lastname: Yamada
authors.forename: T
authors.initials: T
authors.lastname: Hirano
authors.forename: A
authors.initials: A
authors.lastname: Fujimoto
authors.forename: R
authors.initials: R
authors.lastname: Morita
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D002272:Carcinoembryonic Antigen / Q000097:blood; D005260:Female; D005833:Genital Neoplasms, Female / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D005834:Genital Neoplasms, Male / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006801:Humans; D007564:Japan; D008297:Male; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D002272:Carcinoembryonic Antigen