The key to diagnosis of Paget disease (PD) is a high index of suspicion. Paget disease should be strongly considered when any areolar or nipple lesion fails to heal with topical steroid therapy. • Inflammatory breast cancer (IBC) is a clinical diagnosis. Identification of warning signs and clinical symptoms is crucial to prompt diagnosis and appropriate referral. • Occult primary breast carcinoma (OPBC) should be differentiated from lymphoma or non-breast metastatic disease. • The most frequent non-epithelial breast malignancies, even if they are rare, are grade III phyllodes tumour, angiosarcoma and primary breast lymphoma. Future Directions. Within breast oncology, Paget disease, inflammatory breast cancer and occult primary breast cancer should be considered orphan diseases for the heterogeneity of their immunohistochemical profiles and controversial treatments. These diseases affect very few patients, information is as well scarce, and therefore treatment can be difficult to plan. Nowadays, research is moving first meaningful footsteps toward these so far neglected diseases.
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