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

The typical clinical presentation in extramammary Paget's disease consists of multifocal or single lesions that are dry, raised, erythematous, and slow-growing. Over time, the lesions may evolve into crusted, eczematoid, ulcerated, or papillary lesions. Because of their benign appearance, the lesions are often neglected for several years before being presented for medical attention.

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 favorable 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 male genital disease or perianal.
• 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 extramammary Paget's disease (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 adnexa, dermis, or lymph nodes. Both morbidity and mortality are increased in these patients because of the chemotherapy or extensive surgical treatment that they need.
• The prognosis for extramammary Paget's disease (EMPD) heavily depends on early diagnosis with definitive surgical treatment. Full recovery in extramammary Paget's disease is possible in patients with negative margins after micrographic surgery and purely epidermal. One study showed a mortality of 46% for those with underlying carcinoma and 18% for patients without associated carcinoma.
• Of patients with extramammary Paget's disease, approximately 24% have an associated underlying adnexal adenocarcinoma, which has been associated with a worse prognosis, with mortality rates up to 46%. Up to 12% of patients with extramammary Paget's disease have a concurrent internal malignancy that may be located nearby, and this incidence has been found to be greatest in patients with perianal Paget's disease.
• The prognosis is good when the disease is confined to the epidermis. However, in the presence of dermal invasion, the prognosis is poor.
• Poor prognostic factors for extramammary Paget's disease include the following:
  • Perianal disease
  • Dermal invasion
  • Lymph node metastasis
• The recurrence rate of extramammary Paget's disease (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.

References

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