title: Perineal Paget's disease: A rare disorder and review of literature.
abstract: BACKGROUND
  Extramammary Paget's disease (EMPD) occurs commonly in perineum, vulva and perineal region and is considered as a complex disorder due to different clinical and histological features.
  
  
  METHODS
  A 61 years old woman had a dermatologic evaluation for anal itch and underwent a skin biopsy with diagnostic of Paget disease in perianal region. Pelvic magnetic resonance showed a huge tumor which involved skin, derma and gluteus and she firstly refused any surgical treatment. One year later, because of increasing of the tumor and bleeding, she underwent the surgical procedure with a complete excision, resection of the skin of the anus, inguinal lymphadenectomy and left colostomy. Because of lymph nodes metastasis, a VLS Miles was sequentially performed. She started oncological follow up that showed liver, lung and pelvic metastasis. She survived for 24 months after surgery.
  
  
  CONCLUSIONS
  In literature, less than 200 cases of perineal Paget's disease have been reported. EMPD in some cases has an associated adenocarcinoma, which has been associated with a worse prognosis and high mortality. Wrong diagnosis and a superficial consideration of a benign evolution should be considered as the first mistake in clinical practice.
  
  
  CONCLUSIONS
  Our surgical approach is considered in literature as the best one for those cases, followed by the oncological treatment. Those patients need to be better studied and more attention should be paid to the clinical presentation.
authors.affiliation: General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Carbotta
authors.affiliation: General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.
authors.forename: Pierluca
authors.initials: P
authors.lastname: Sallustio
authors.affiliation: General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Prestera
authors.affiliation: General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.
authors.forename: Rita
authors.initials: R
authors.lastname: Laforgia
authors.affiliation: General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Lobascio
authors.affiliation: General Surgery Unit, Department of Emergency and Organ Transplantation-University of Bari, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Palasciano
keywords: Extramammary Paget's disease; Perineal Paget's disease