Vulvar Paget Disease: One Century After First Reported.

OBJECTIVES.: To provide a critical assessment of the published literature on vulvar Paget disease and to allow individualized approaches to affected patients. MATERIALS AND METHODS.: A computerized search for studies published in the literature up to June 2002 was carried out using Ovid(c) and Medline databases. We excluded single case reports, letters to editors, and abstracts. RESULTS.: Historical and epidemiological aspects of vulvar Paget disease are summarized. Clinical and histopathological data support a recent proposal to classify vulvar Paget disease into two categories, primary and secondary, with significant clinical and prognostic implications. The treatment for primary vulvar Paget disease is wide and deep surgical excision. Inguinofemoral lymphadenectomy is added in the management of invasive neoplasms. In the presence of secondary Paget disease, therapy must be directed toward treatment of associated carcinoma. CONCLUSIONS.: The subclassification of vulvar Paget disease is essential for correct clinical management and treatment. Immunohistochemistry may assist in this important distinction.