title: Photodynamic therapy with M-ALA as non surgical treatment option in patients with primary extramammary Paget's disease.
abstract: BACKGROUND
  Extramammary Paget disease (EMPD) is a rare neoplasm of the skin that presents with erythematous or leukoplacic plaques causing pruritus and pain. Standard treatment is surgical but local failures and recurrences are frequent, leading to multiple mutilating surgeries. Aim of the study is to evaluate the effectiveness of photodynamic therapy (PDT) to obtain a clinical response and symptom control with a non surgical approach in these patients.
  
  
  METHODS
  After disease extension evaluation and symptoms assessment women with EMPD were prospectively treated with aminolevulinic-acid methyl-esther (M-ALA) PDT. Clinical and symptoms response were evaluated after 3 cycles and after any further PDT.
  
  
  RESULTS
  Thirty-two patients with vulvar EMPD underwent M-ALA PDT. In sixteen (50%) patients the lesion extended to the perineal and/or perianal area. After three courses of treatment, three patients (9.4%) had a complete resolution of the symptoms; 25 patients (78.1%) a partial resolution and a stable disease was recorded in four patients (12.5%). None of the patients had progression of disease. Both size of the lesion and EMPD associated symptoms decreased significantly after three courses of treatment. Eighteen patients (56.2%) recurred and 16 (88.9%) were treated with further PDT. Among the 26 patients who underwent a further PDT, 16 patients (61.5%) achieved at least a partial response.
  
  
  CONCLUSIONS
  M-ALA PDT even if not curative is a reliable strategy to control EMPD and its associated symptoms even in an outpatient setting. M-ALA PDT is able to control large and multiple lesions regardless of the area involved, preserving cosmetic and/or functional anatomy.
authors.affiliation: Department of Gynecologic Oncology, IRCCS -Istituto Nazionale Tumori di Milano, via Giacomo Venezian 1, Milano, Italy. rosanna.fontanelli@istitutotumori.mi.it
authors.forename: Rosanna
authors.initials: R
authors.lastname: Fontanelli
authors.forename: Andrea
authors.initials: A
authors.lastname: Papadia
authors.forename: Fabio
authors.initials: F
authors.lastname: Martinelli
authors.forename: Domenica
authors.initials: D
authors.lastname: Lorusso
authors.forename: Barbara
authors.initials: B
authors.lastname: Grijuela
authors.forename: Marina
authors.initials: M
authors.lastname: Merola
authors.forename: Eugenio
authors.initials: E
authors.lastname: Solima
authors.forename: Antonino
authors.initials: A
authors.lastname: Ditto
authors.forename: Francesco
authors.initials: F
authors.lastname: Raspagliesi
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000622:Aminolevulinic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000188:drug therapy*; D010778:Photochemotherapy / Q000379:methods*; D017319:Photosensitizing Agents / Q000627:therapeutic use*; D011446:Prospective Studies; D014846:Vulvar Neoplasms / Q000188:drug therapy*
chemical_list: D017319:Photosensitizing Agents; C114767:delta-aminolevulinic acid methyl ester; D000622:Aminolevulinic Acid