A review of extramammary paget�?¢�?�?��?�?�s disease: Clini | 17861
International Research Journals

A review of extramammary paget�?¢�?�?��?�?�s disease: Clinical presentation, diagnosis, management and prognosis


Ambrogio P. Londero, Serena Bertozzi, Stefania Salvador, Arrigo Fruscalzo, Vito D’Aietti, Tiziana Grassi, Lorenza Driul, Laura Mariuzzi, Diego Marchesoni and Ralph J. Lellé

Extramammary Paget’s Disease (EMPD) is a rare neopl astic lesion, which represents less than 1% of vulvar neoplasms. The lesion generally appears as e czema and the most frequently reported symptom is the itch. Also because of these poor clinical fe atures, there is usually a delay in its diagnosis, based on the typical biopsy histological pattern. It has a good prognosis in absence of malignancy, but can result in a heavy quality of life impairment becaus e of frequent recurrence with necessity of ablative therapies and anxiety for possible cancerization. R arely EMPD can be invasive or associated to adenocarcinoma or other kinds of cancer. The first choice therapy is the surgical excision, with inguinal lymphadenectomy in case of infiltrative di sease. However, many other conservative therapies, including the topical use of antiblastic, immuno-mo dulating, and hormone-modulating drugs, are used against EMPD even if still off label. After complet ion of this article, the reader should be able to r ecall the clinical manifestations of the EMPD, the histol ogical pattern which allows its diagnosis, and to s tate the options for a treatment, which should be conser vative and at the same time as radical as possible.

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