Mammary Paget Disease Treatment & Management

Updated: May 14, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Surgical Care

Mastectomy (radical or modified) and lymph node clearance are appropriate therapies for patients with mammary Paget disease (PD) with a palpable mass and underlying invasive breast carcinoma. [28, 34] As many as two thirds of patients are reported to have axillary lymph nodes positive for metastasis. Noninvasive breast carcinoma (in situ carcinoma) is found in about 65% of patients with mammary PD without a palpable mass.

Conservative management includes a combination of local excision of the nipple, wedge resection of the underlying breast, and radiation therapy. The number of patients treated by one or more conservative measures (eg, nipple excision and wedge excision of the underlying breast, cone excision, radiation therapy) is small. Breast conservation therapy with lumpectomy and radiation is an effective option, compared with mastectomy, for patients with Paget disease–ductal carcinoma in situ and Paget disease–invading ductal carcinoma. [35]

Patients who underwent cone excision and elective tamoxifen therapy had recurrences after an average follow-up of 4.6 years; some developed metastases. Therefore, cone excision is not sufficient therapy for patients with disease limited to the nipple.

Wide local excision with axillary node sampling is recommended for patients with or without a clinical mass.

Radiation therapy alone does not always control occult breast cancer; however, it may be used for patients who refuse mastectomy or those who are medically unfit for surgery.