disease free at follow-up (9–88 months). Larger series are needed to firmly establish the role of PDT in the treatment of non-invasive EMPD.

22.7.3 Laser Vaporization

Destruction of EMPD with carbon dioxide laser vaporization has been documented. While treatment of vulvar EMPD with laser vaporization preserves vulvar anatomy, there is a significant association with postoperative pain and a high recurrence rate [11].

22.7.4 Radiation Therapy

Radiation therapy has been used as a treatment for primary EMPD, following surgical excision of EMPD with the aim of reducing local recurrence, and as a treatment for recurrent disease. Intraepidermal and invasive EMPD have both been treated with radiation therapy, primarily disease located in the anogenital region. Patients in published series have generally been considered poor surgical candidates, and therefore, recurrence rates likely reflect a higher risk subset of patients with a worse prognosis.

Radiation therapy has proven to have acceptable cure rates for primary EMPD. Patients with secondary EMPD have an extremely poor prognosis, regardless of treatment with radiation therapy. In a 2002 review of the existing literature on radiation therapy for perianal Paget’s disease, 43 cases were analyzed [34]. The overall outcome was progressive disease in 56%, no sign of recurrent disease in 37%, and unknown in three cases (7%). Of the patients with primary EMPD, recurrence after radiation therapy was 35%, compared to a recurrence of 77% in patients with secondary EMPD. In this review, 5-year survival was 20% for invasive perianal EMPD and 94% for non-invasive PPD, demonstrating a statistically significant difference in the survival curves.

Typical regimens consist of treating a field including 3 cm surrounding visible disease with 40–50 Gy. Photon, electron, and brachytherapy have all been used with success. Lower doses of radiation therapy are associated with a higher risk of recurrence, however [35, 36].

Radiation therapy has been successful in treating EMPD of the vulva, in many cases leading to a recur- rence-free state [12]. Radiation therapy, like topical

treatments, is most applicable in patients who are unsuitable candidates for surgical intervention. In cases where surgery for intraepidermal disease would result in significant loss of function (abdominoperineal resection and colostomy formation), reserving surgery for salvage treatment for cases of radiation therapy failure may be an acceptable alternative [34]. Chronic radiation toxicity is a potential concern, with pigmentary changes and atrophy as noted complications.

22.7.5Chemotherapy for EMPD: Local and Systemic

Systemic chemotherapy has shown little efficacy in the treatment of metastatic disease. 5-fluorouracil, docetaxel, and cisplatin-based chemotherapy have been used in multiple patients with mixed results [35, 36].

Axillary EMPD has been treated with local chemotherapy using doxorubicin incorporated in large liposomes and paclitaxel incorporated in albumin nanoparticles [37]. Complete cure has not been obtained, but improved quality of life has been achieved with minimal treatment-related morbidity.

Summary: Conclusion

•EMPD most commonly presents as an intraepidermal adenocarcinoma with indistinct margins, affecting genital skin.

•EMPD is reported to sometimes occur in association with internal malignancy, though the true relationship is unclear in many cases.

•Distinguishing underlying adnexal carcinoma from spread of epidermal disease may be difficult.

•Mohs surgery is effective in treating EMPD. The addition of CK7 staining shows promise in improving the accuracy of margin control and reducing recurrence rates.

22.8Conclusion

EMPD is an intraepidermal adenocarcinoma most often limited to the epidermis, with typical cases affecting genital skin in women and men. In patients with invasive disease, prognosis is based on the degree of invasion,

with tumors less than 1 mm deep having very low mortality. Careful evaluation for an underlying malignancy should be carried out to exclude life-threatening disease, but the association of many coexistent malignancies is controversial. Reported underlying adnexal adenocarcinoma may, at least in some patients, represent invasive spread of primary epidermal disease. Mohs surgery has proven effective in the treatment of EMPD, and the implementation of CK7 shows promise in further reducing the recurrence rate. Alternative treatments, including topical treatment with imiquimod and 5-fluorouracil as well as photodynamic therapy, may be effective in select cases of EMPD.

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