VACO

Background

Paget disease of the vulva is rare. It occurs in women in the seventh decade of life but can occur in young patients. Symptoms of pruritus and tenderness or identification of a vulvar lesion occur most frequently. The patient may experience symptoms for several years before seeking medical advice. The lesion may be localized to one labium or involve the entire vulvar epithelium. It may extend to the perirectal area, buttocks, inguinal area, or mons. It has been observed extending into the vagina. Because of its rarity, frequency data are unavailable.

Clinical and histological features

Vulvar lesions usually are hyperemic, and they may be demarcated sharply and thickened with foci of excoriation and induration. The vulvar skin may be thick, leading to the impression of leukoplakia with the cake icing effect. This classic finding almost is pathognomonic for Paget disease. The lesion is usually superficial and is considered an intraepithelial lesion, although an underlying adenocarcinoma may be associated with Paget disease. Older literature suggests that underlying adenocarcinoma occurs in about a quarter of cases, but more recent data find less association of Paget disease with an underlying adenocarcinoma.

Obtain adequate biopsies to make an accurate diagnosis. Histologically, it commonly presents with large cells of clear cytoplasm in a heavy lymphocytic infiltration in the dermis; it can be confused with a melanotic melanoma. If any thickened indurated area is present, then obtain adequate deep biopsy in order to rule out adenocarcinoma.

Treatment

If only intraepithelial Paget disease is present, wide local excision is adequate treatment. Histological evaluation of the epithelium extends, in many instances, far beyond the visual limits of the lesion, and, therefore, wide adequate margins are necessary to remove the lesion. If an underlying adenocarcinoma is present, then treat the lesion as invasive squamous cell carcinoma is treated. If tumor cells are present at the margin of the excision, then recurrence can be quite high. Some investigators obtain frozen section of the margins and, if positive, continue to obtain wider margins. Unfortunately, even with negative margins, recurrences are possible, and new lesions can be treated in the same manner as the primary disease (ie, wide local excision). These may occur years after diagnosis of the primary lesion.

Currently, intraepithelial Paget disease and Paget disease with an underlying adenocarcinoma are thought to be 2 separate entities. The cases of patients who were diagnosed with Paget disease and who refused primary treatment have been followed for over a decade without development of an underlying adenocarcinoma. As with other vulvar lesions, frequent visual examinations are necessary in order to determine disease-free status.

http://www.emedicine.com/med/topic3296.htm#section~PagetDisease

My Gynaecologist thinks that I may have Paget's Disease of the Vulva.What is this?