Osther P J, Balslev E, Blichert-Toft M
Surgical Department K, University Hospital, Odense, Denmark.
Acta Chir Scand. 1990 May;156(5):343-52.
Paget's disease of the nipple, although recognized since 1874, remains in several respects enigmatic. The two main theories of its origin are 1) epidermotropic, i.e. ductal cancer cells migrating to the epidermis of the nipple, and 2) in situ appearance of malignant keratinocytes expressing the multicentricity of breast cancer. The literature is reviewed. Clinical, histologic (including classification), histochemical and electron microscopy observations and diagnostic considerations are discussed. Diagnosis is often delayed, with adverse consequences for treatment. The nipple lesions may be accompanied by ductal carcinoma of local or more extensive in situ type, or invasive tumour. Lymph-node metastasis seems to be the most important prognostic factor. The merits of radical vs. modified radical mastectomy and local excision, with or without adjuvant radiotherapy, are considered. Major studies of breast-conserving management of Paget's disease of the nipple are in progress.
乳头佩吉特病虽自1874年就已被认识，但在多个方面仍存在谜团。其起源的两种主要理论为：1）表皮趋向性，即导管癌细胞迁移至乳头表皮；2）表达乳腺癌多中心性的恶性角质形成细胞原位出现。本文对相关文献进行了综述。讨论了临床、组织学（包括分类）、组织化学及电子显微镜观察结果和诊断要点。诊断往往延迟，对治疗产生不利影响。乳头病变可能伴有局部或更广泛原位型导管癌或浸润性肿瘤。淋巴结转移似乎是最重要的预后因素。文中考虑了根治性乳房切除术与改良根治性乳房切除术以及局部切除术（有无辅助放疗）的优缺点。关于乳头佩吉特病保乳治疗的主要研究正在进行中。
