title: Cytology of extramammary Paget's disease of the vulva. A case report.
abstract: BACKGROUND
  Paget's disease of the vulva (PDV) is a rare entity, with only a few cytologic descriptions having been published on it. Diagnosis is usually delayed because it is often clinically mistaken for some types of dermatosis, and biopsy is usually postponed.
  
  
  METHODS
  A 56-year-old woman presented with a pruritic, erythematous and ulcerated superficial lesion on the right labium majus of approximately eight months' duration. A vulvar cytologic smear showed a bloody and inflammatory background with many single malignant cells; scarce malignant cell aggregates; and abundant, mature squamous and dyskeratotic cells. The tumor cells were large, with a frequently eccentric, large nucleus. Some binucleated forms were noted. Nucleoli were rare. Cytoplasm varied from pale and delicate to densely basophilic. Intracytoplasmic vacuoles were very rare. Tumor cell aggregates were small and exhibited pseudocannibalism. Short strands of malignant cells arranged in an Indian file pattern were also evident. Histologic examination of a wedge biopsy, wide local excision of the lesion and simple vulvectomy showed PDV.
  
  
  CONCLUSIONS
  Knowledge of the cytologic features of PDV could provide a highly probable cytologic diagnosis of the disease and should alert the clinician to the need for immediate biopsy. Systematic collecting of smears from any eczematous change in the vulva should be considered a first step to early diagnosis of malignancy.
authors.affiliation: Pathology Department, Hospital Universitario de Valme (Servicio Andaluz de Salud), Seville, Spain.
authors.forename: Victor Manuel
authors.initials: VM
authors.lastname: Castellano Megías
authors.forename: Carolina
authors.initials: C
authors.lastname: Ibarrola de Andrés
authors.forename: Diego
authors.initials: D
authors.lastname: Martínez Parra
authors.forename: Isabel
authors.initials: I
authors.lastname: Lara Lara
authors.forename: Carmen
authors.initials: C
authors.lastname: Pérez Palacios
authors.forename: José María
authors.initials: JM
authors.lastname: Conde Zurita
mesh_terms: D005260:Female; D006801:Humans; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000473:pathology*; D014844:Vulva / Q000473:pathology; D014845:Vulvar Diseases / Q000473:pathology*