Abstract
Neuroendocrine breast cancer (NEBC) is a rare histotype of breast carcinoma that presents, in most cases, positive hormone receptors and negative HER2. Indeed, the analysis of gene expression profiles revealed that NEBC belongs mainly to the luminal subtype. Cases of HER2-positive and triple-negative NEBC are rare. The cardinal treatment of early NEBC is surgery, similar to the treatment of invasive non-special histological type carcinoma. The use of radiotherapy follows the criteria applied in infiltrating breast cancer of non-special histotype. In the post-operative phase, therefore after the surgical treatment of mammary quadrantectomy, or mastectomy associated with homolateral sentinel lymph node removal ± axillary dissection, based on the histopathological characteristics of the tumor, the use of chemotherapy (anthracycline + taxane) and/or hormone therapy, whether or not associated with anti-HER2 therapy (trastuzumab) is the rule. Literature data report the use of cisplatin and etoposide, as in small cell lung cancers. Most of the information currently available derive from single case reports or a series of clinical cases; it follows the difficulty of formulating definite recommendations on the correct management of this histological type of breast cancer. This review describes available knowledge on this rare entity to improve the diagnostic and therapeutic strategies and offer insights to stimulate exploration of the many aspects still unknown.
Highlights
This review describes available knowledge on this rare entity to improve the diagnostic and therapeutic strategies and offer insights to stimulate exploration of the many aspects still unknown
Breast cancer is the primary cause of death among Western women [1] and primary neuroendocrine breast cancers (NEBCs) are a rare subtype of breast cancer [2] that, according to the World Health Organization (WHO), represent 2–5% [3,4]
Neuroendocrine differentiation is observed in up to 20% of mammary carcinomas, so the real incidence of NEBCs is difficult to evaluate because immunohistochemical neuroendocrine markers are not usually used in the diagnosis of breast cancer [5]
Summary
Breast cancer is the primary cause of death among Western women [1] and primary neuroendocrine breast cancers (NEBCs) are a rare subtype of breast cancer [2] that, according to the World Health Organization (WHO), represent 2–5% [3,4]. Neuroendocrine differentiation is observed in up to 20% of mammary carcinomas, so the real incidence of NEBCs is difficult to evaluate because immunohistochemical neuroendocrine markers are not usually used in the diagnosis of breast cancer [5]. The most recognized theory on the histogenesis of NEBC suggests its derivation from the divergent differentiation of a neoplastic stem cell in both epithelial and neuroendocrine cells [6,7]. Another theory hypothesizes a derivation from neural crest cells that migrate to the mammary glands or an origin from neuroendocrine cells present in breast tissue [8]. SepreecxifpicrehsissitoonlogoifcHalEtyRp2es[1m2]a.yInprNesEenBtCs, a lonweuirnoceinddeonccreinoefdmififcerroecnatilactifiiocnatsiuocnhsahsahsybpeeerncerleluploarrtendeu[r1o3e]n. dSopcerciinfiecchairsctionloomgiacaanl tdyspoelsidm-paayppillraersyent neucarroceinndomocari[n13e]d. ifferentiation such as hypercellular neuroendocrine carcinoma and solid-papillary carcinoma [13]
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