Management of Neuroendocrine Breast Carcinoma (NEBC): Review of Literature

Abstract

Extra pulmonary high-grade poorly differentiated neuroendocrine carcinomas (EP-NECs) are rare tumors that usually arise in the gastrointestinal and genitourinary tracts. Primary neuroendocrine carcinoma of the breast (NEBC) is extremely rare, representing less than 0.1% of all breast cancers and less than 1% of neuroendocrine neoplasms. Consequently, they can be misdiagnosed as other types of breast cancer, however, proper immunohistochemical (IHC) studies can assist with making the correct diagnosis. Management of NEBC can be challenging given the paucity of evidence-based literature and should not routinely follow the therapeutic guidelines of other breast cancers. In this article, we review the current literature regarding the management of NEBC.

Keywords: breast; carcinoma; management; neuroendocrine; review.

FIGURE 1

Small cell neuroendocrine carcinoma of the breast. (A) H&E staining on high power field (×40) showing neoplastic cells with nuclear pleomorphism, nuclear molding, mitotic figures, hyperchromatic nuclei, minimal cytoplasm, and indistinct nuclei consistent. (B) Immunohistochemical staining positive for synaptophysin [14].

FIGURE 2

Large cells neuroendocrine carcinoma of the breast. (A) H&E stain, ×10, and (B) on immunohistochemistry, tumor cells show diffuse positive stain for Chromogranin A (×10) [6].

FIGURE 3

Treatment algorithm for Neuroendocrine Breast Carcinoma (NEBC): EP, Etoposide plus platinum (cisplatin or carboplatin); TC, Taxotere and cyclophosphamide; IC, Irinotecan plus cisplatin; ICPIs, Immune check point inhibitors.