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Neuroendocrine neoplasm of the breast (bNENs) are considered a rare disease, even if inWHO data they represent about 2-5 % of all breast cancer. The last WHO classification includes: welldifferentiated neuroendocrine tumor (bNET), neuroendocrine carcinoma (NEC) and invasive carcinomawith neuroendocrine differentiation. The current knowledge on clinical management of bNENs is poor andpatients are usually treated according to non-endocrine tumor components guidelines.Materials and Methods: We presented our experience of six cases of bNENs. Moreover, we conducted asystematic review of published data on diagnosis, treatment and outcome of this kind of tumors.Results: bNENS usually presented as palpable breast masses, classically appearing as irregular hypoechoiclesions at US examination and as hyperdense masses at mammography. Usually pre-operative tumor biopsyis not able to recognize the neuroendocrine components and the final diagnosis is performed only ondefinitive histopathological assessment. The most frequent subtype seems to be neuroendocrine carcinomaand synaptophysin is positive in most specimens. Treatment strategies, including surgical treatment,radiotherapy and medical treatment are nowadays based on current non-endocrine breast cancer guidelines,independently from neuroendocrine components, even if some studies have proposed the use of somatostatinanalogues for bNET and cisplatin-etoposide for NEC. Prognosis of all bNENs, especially of poorlydifferentiated neoplasia, seems worse compared to non-neuroendocrine breast cancer and stage andmorphology seem the best predictor of tumor outcome.Conclusions: We provide an algorithm for clinical management of bNETs, basing on available data. Morestudies are necessary for confirming the best treatment strategy for these patients, in order to improveclinical outcome

Diagnosis and Clinical Management of Neuroendocrine Tumor of the Breast: Report of Six Cases and Systematic Review of Existing Literature