Introduction: Neuroendocrine tumors are rare neoplasms that can occur in various organs, being most common in the gastroenteropancreatic tract and lungs. Neuroendocrine carcinoma of the breast (NECB) is a rare and heterogeneous subtype, lacking standardized therapeutic guidelines and protocols, with limited prospective clinical studies. This paper reviews the available literature and presents a clinical case to illustrate the diagnostic and therapeutic management of NECB. Methods: A literature review was conducted using databases such as PubMed, Medline, and Google Scholar to identify studies on NECB. Additionally, a clinical case of a patient diagnosed with NECB was analyzed, documenting her clinical presentation, diagnosis, treatment, and evolution. Results: NECB represents less than 1% of all breast cancers, with variability in its presentation and prognosis. The case of a patient with NECB who received systemic treatment based on chemotherapy is presented, exemplifying the management of this rare and diagnostically and therapeutically challenging tumor. Discussion: The diagnosis of NECB is complex due to the rarity of the tumor and the lack of specific clinical characteristics. Immunohistochemistry is crucial for confirming the diagnosis. The lack of standardized therapeutic guidelines complicates treatment, often necessitating the extrapolation of strategies from other invasive breast cancers. Conclusion: NECB is a rare and aggressive tumor with limited treatment options. Chemotherapy with cisplatin and etoposide can be effective, as observed in this case. Given the aggressive nature of this tumor and the few treatment options available, it is vital to consider participation in clinical trials to improve outcomes. Additionally, a multidisciplinary approach and evaluation by a tumor board are essential to guide therapeutic decisions and ensure the best possible care for these patients.
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Camejo et al. (2024) studied this question.
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