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We present here a case of a 35-year-old woman diagnosed with primary neuroendocrine carcinoma of the breast (NEC - small cell type. We discuss the importance of histological and molecular criteria for primary neuroendocrine mammary neoplasm, established by the World Health Organization in 2003 and 2012. We present information about differential diagnosis, prognostic factors, surgical treatment, adjuvant treatment, and novel therapies for primary NECB. Although this disease is not so uncommon but it is highly underreported and with co
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