NCCN VERSION 2 2015

The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®.

MS-58

NCCN Guidelines Index

Breast Cancer Table of Contents

Discussion

NCCN Guidelines Version 2.2015

Breast Cancer

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

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