NCCN VERSION 2 2015

NCCN Guidelines Version 2.2015 Breast Cancer

NCCN Guidelines Index Breast Cancer Table of Contents Discussion

There are no category 1 data that specifically address local management of Paget’s disease. Systemic therapy is based on the stage and biological characteristics of any underlying cancer, and is supported by the evidence cited in the relevant stage-specific breast cancer treatment guidelines. Management of Paget’s disease has traditionally been total mastectomy with axillary dissection. Total mastectomy remains a reasonable option for patients regardless of the absence or presence of an associated breast cancer. 518 Data demonstrate that satisfactory local control may be achieved with breast-conserving surgery including the excision with negative margins of any underlying breast cancer along with resection of the NAC followed by whole breast radiation therapy. 521-525 The risk of ipsilateral breast recurrence after breast-conserving NAC resection and radiation therapy with or without an associated cancer is similar to that with breast-conserving surgery and radiation therapy with the typical invasive or in situ cancer. For Paget’s disease without an associated cancer (ie, no palpable mass or imaging abnormality), it is recommended that breast-conserving surgery consist of removal of the entire NAC with a negative margin of underlying breast tissue. In cases with an associated cancer elsewhere in the breast, the surgery includes removal of the NAC with a negative margin, and removal of the peripheral cancer using standard breast-conserving technique to achieve a negative margin. It is not necessary to remove the NAC and the peripheral cancer in continuity in a single surgical specimen or through a single incision. Mastectomy also remains an appropriate treatment option. ALN staging is not necessary when breast-conserving therapy is used to treat Paget’s disease with underlying DCIS without evidence of invasive cancer following clinical examination, imaging evaluation, and

provides a table outlining general recommendations for the frequency and type of monitoring as a baseline before initiation of new therapy, for monitoring the effectiveness of cytotoxic chemotherapy and endocrine therapy, and assessment when there is evidence of disease progression. The panel has indicated in a footnote that the frequency of monitoring can be reduced in patients who have long-term stable disease. These are guidelines and should be modified for the individual patient using clinical judgement, especially in those with stable or responding disease for long periods of time. Special Situations Paget’s Disease Paget’s disease of the breast is a rare manifestation of breast cancer characterized by neoplastic cells in the epidermis of the NAC. 516 It most commonly presents with eczema of the areola, bleeding, ulceration, and itching of the nipple. The diagnosis is often delayed because of the rare nature of the condition and confusion with other dermatologic conditions. There is an associated cancer elsewhere in the breast in up to about 80% to 90% of cases. 517-519 The associated cancers are not necessarily located adjacent to the NAC and may be either DCIS or invasive cancer. Women with clinical signs that raise suspicion for Paget’s disease require a complete history and physical examination and diagnostic breast imaging. Any breast lesion identified by imaging or examination should be evaluated according to the NCCN Guidelines for Breast Screening and Diagnosis . The skin of the NAC should undergo surgical biopsy, including the full thickness of the epidermis including at least a portion of any clinically involved NAC. When biopsy of the NAC is positive for Paget’s disease, breast MRI is recommended to define the extent of disease and identify additional disease. 519,520

Version 2.2015, 03/11/15 © National Comprehensive Cancer Network, Inc. 2015, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. MS-58