NCCN VERSION 2 2015

NCCN Guidelines Version 2.2015 Breast Cancer

NCCN Guidelines Index Breast Cancer Table of Contents Discussion

diagnosis of IBC. 9,572 The differential diagnosis includes cellulitis of the breast and mastitis. In the past, IBC has often been placed under the general heading of locally advanced breast cancer. There is a growing body of evidence that IBC patients, when compared with noninflammatory forms of locally advanced breast cancer, are more likely to have a less favorable prognosis 573-575 and to be younger at the time of disease presentation. 576 Hormone receptor-positive IBC is associated with a slightly more favorable prognosis, 570,577 whereas HER2 overexpression in IBC is associated with a poor prognosis. 570,578 The NCCN Panel acknowledges that studies focusing on genetic characterization of IBC are needed to more clearly define IBC as a disease entity and to optimize treatment. 579,580 Nevertheless, current evidence provides justification for a separate guideline for the workup and treatment of patients diagnosed with IBC. Women with a clinical/pathologic diagnosis of IBC without distant metastasis (stage T4d, N0-N3, M0) should undergo a thorough staging evaluation by a multidisciplinary team. Recommendations for workup include a complete history and physical examination involving a CBC and platelet count. A pathology review and pre-chemotherapy determinations of tumor hormone receptor and HER2 receptor status should be performed. HER2 has a predictive role in determining which patients with IBC will benefit from HER2 targeted therapy. The NCCN Panel endorses the CAP protocol for pathology reporting ( www.cap.org ) and endorses the StageT4d, N0- N3, M0 Workup

Endocrine therapy and radiation therapy are contraindicated during pregnancy. Endocrine therapy and radiation therapy, if indicated, should thus not be initiated until the postpartum period. Communication between the oncologist and maternal fetal medicine specialist is essential at every visit and for every treatment decision point for the patient. Inflammatory Breast Cancer Inflammatory breast cancer (IBC) is a rare, aggressive form of breast cancer estimated to account for 1% to 6% of breast cancer cases in the United States. 566,567 IBC is a clinical diagnosis that requires erythema and dermal edema (peau d’orange) of a third or more of the skin of the breast with a palpable border to the erythema. IBC is usually hormone receptor-negative and is more frequently HER2-positive than the usual ductal breast cancers. Studies on gene expression profiling of IBC have demonstrated that all the subtypes of IBC exist, but basal and HER2 overexpressed are more frequent. 568-571 According to the 7 th edition of the AJCC Cancer Staging Manual , IBC is classified as stage IIIB, stage IIIC, or stage IV breast cancer, depending on the degree of nodal involvement and whether distant metastases are present. The primary tumor of IBC is classified as T4d by definition, even when no mass is specifically apparent in the breast. On radiographic imaging, findings of skin thickening and, in some cases, an underlying mass are observed. Despite use of the term “inflammatory,” the characteristic clinical features of IBC are due to blockage of dermal lymphatics by tumor emboli. Although a biopsy is required to evaluate for the presence of cancer in breast tissue and the dermal lymphatics, a diagnosis of IBC is based on clinical findings, and dermal lymphatic involvement is neither required, nor sufficient by itself, to assign a

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-62