Paget’s disease

11 May 2012, by RENAUD-VILMER C.

Cutaneous Paget’s disease is an intra-epidermal adenocarcinoma which is characterized by the presence of large cells with a large nucleus, called Paget cells, with immunohistochemical features of glandular cells. These diseases are rare and though the clinical, histologic and immunohistochemical features are well defined, the causes are still uncertain. A distinction should be made between mammary Paget’s disease (MPD) and extramammary Paget’s disease (EMPD). MPD is almost always associated with an underlying breast adenocarcinoma, which is what makes it a serious dermatological condition, while EMPD is only associated with an underlying adenocarcinoma in 2-20% of cases. However, in 9-30% of patients EMPD could be associated with an underlying adenocarcinoma, and could also progress by itself into an invasive adenocarcinoma. However, these lesions are slow-growing, which allows preventive treatment to be instituted if the diagnosis is made in time. 

The clinical appearance of these lesions may be misleading. They usually present as a red, scaly lesion which may be itchy, and which gradually enlarges. Diagnosis is based on histologic examination which requires a skin biopsy.


The pretreatment managment looks for an underlying breast carcinoma (mammography, breast ultrasound and breast MRI if necessary).

The standard treatment consists of modified radical mastectomy with sentinel node biopsy, due to the fact that MPD is almost always associated with an underlying carcinoma and the presence of the sentinel node. If the patient refuses mastectomy and in the absence of a radiologically visible underlying carcinoma, a wide excision of the nipple with a retro-areolar cone excision and sentinel node biopsy is proposed. If the margins are negative, adjuvant breast irradiation is proposed. For positive margins, mastectomy is again proposed. Follow-up care depends on the staging of the underlying cancer.


The pretreatment managmentshould include a clinical examination for invasiveness or underlying adenocarcinoma. Any infiltrated area, lump or ulceration should be biopsied. A distant adenocarcinoma should be looked for, even though some authors believe that this association is due to an effect of age (the mean age of patients is generally around 60 years). The workup depends on the anatomic location of the EMPD and includes mammography, genital examination (with pelvic ultrasound) for vulvar locations; urologic examination looking for genitourinary cancer for EMPD of the male genitalia; gastrointestinal workup (clinical, endoscopy, radiology) to look for adenocarcinoma mainly of the lower gastrointestinal tract. If the EMPD is invasive or associated with an underlying adenocarcinoma, MRI of the affected organ (to better visualize local extension), ultrasonography of draining lymph nodes, whole-body CT scan and PET scan are proposed. 

The reference treatment is surgical excision, because only a histologic examination of the complete lesion can rule out invasiveness. New techniques in plastic surgery and anesthesia have facilitated surgical intervention by minimizing postoperative complications and can be used in weak, elderly patients. Unfortunately, local recurrences are very common (17-38% of cases), even when the margins are clear, because these lesions progress by hopping around. Currently, an initial excision with 1 cm margins, with no mutilating result, is most commonly used, since the prognosis of EMPD is good when there is no invasion or underlying adenocarcinoma. Mohs surgery does not protect against recurrences either (11.4-27% recurrence rate). If the excision margins are positive but histology has ruled out invasive EMPD or underlying adenocarcinoma, repeat surgery may be proposed, or else simple clinical monitoring (twice a year for the first three years, then annually), since recurrence only occurs in 50-70% of cases. Other treatments such as phototherapy and Imiquimod are under evaluation but appear to be effective mainly on small lesions. CO2 laser therapy is painful and has a higher recurrence rate than surgery. Radiotherapy can be used in patients whose lesions are very painful due to their extension and whose life expectancy does not exceed 3-5 years. Because radiotherapy may cause chronic radiation dermatitis and does not prevent recurrences after 3-5 years. None of these nonsurgical treatments can be used to look for a zone of invasion within the lesion.

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