Paget's disease of the breast is a rare condition that is associated with underlying breast cancer in the majority of patients. The conventional treatment for Paget's disease has been mastectomy, but there is an increasing trend to consider breast-conserving surgery (BCS) in selected patients. Owing to the uncommon nature of the disease, research studies tend to be small and retrospective. This systematic review presents the published evidence regarding BCS for patients with Paget's disease with a focus on patient selection and oncological safety.

A search of Ovid and PubMed databases was conducted to identify all papers published regarding BCS for Paget's disease.

The search identified 172 papers of which 43 were clinically relevant. BCS is a safe alternative to mastectomy, provided a clear surgical margin is achieved and adjuvant radiotherapy used. However, patients with Paget's disease should be assumed to have underlying breast cancer, and these cancers tend to have poor biological profiles. When BCS is considered, careful preoperative investigation should be undertaken to identify the presence and extent of an underlying cancer. These cancers can be mammographically occult, multifocal or multicentric. Although the evidence is limited, there may be a role for MRI in selecting patients with Paget's disease for BCS.

Patients with Paget's disease are candidates for breast conservation with appropriate preoperative investigations. Oncological outcomes are equivalent to those of mastectomy if surgical margins are achieved and adjuvant radiotherapy is given.