Extramammary Paget's disease natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Natural History
Complications
Prognosis
- The prognosis for primary Extramammary Pagets's disease (EMPD) confined to the epidermis is excellent. However, invasive primary extramammary Paget's disease (EMPD) carries a poor prognosis, particularly if lymphovascular invasion is present.
- The depth of invasion is an important prognostic factor, with microscopic invasive disease (less than 1 mm dermal invasion) having a more favourable prognosis than lesions showing deeper invasion.
- The prognosis decreases substantially with lymphovascular involvement, with a five-year survival rate of 0% in the presence of inguinal lymph node metastases.
Lengthy follow up is advocated in all cases of primary extramammary Paget's disease (EMPD) and each patient should be thoroughly investigated to rule out an underlying malignancy, particularly in cases of perianal or male genital disease. The prognosis of secondary extramammary Paget's disease (EMPD) depends on the prognosis of the underlying carcinoma but is generally worse than that for primary EMPD.
The course of EMPD may extend over a period of 10-15 years without evidence of cancer or metastases. In a minority of patients, tumor cells infiltrate the dermis, adnexa, or lymph nodes. Both mortality and morbidity are increased in these patients because of the extensive surgical treatment or chemotherapy that they need.
The prognosis for EMPD heavily depends on early diagnosis with definitive surgical treatment. Full recovery is possible in patients with purely epidermal disease and negative margins after micrographic surgery. One study showed a mortality of 18% for patients without associated carcinoma and 46% for those with underlying carcinoma.
Perianal disease, dermal invasion, and lymph node metastasis are poor prognostic indicators. The recurrence rate of EMPD is 30%, even with margin control. The average time to recurrence is 2.5 years, with case reports of more than 10 years follow-up.
The prognosis of extramamary Paget's disease depends on the following:
- Whether or not the tumor can be removed by surgery
- The stage of the cancer: the size of the tumor, whether the cancer has spread outside the vulva or penis
- The patient’s general health
- Whether the cancer has just been diagnosed or has recurred