Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2020 Jul 25;37(8):70.
doi: 10.1007/s12032-020-01396-4.

Neuroendocrine breast carcinoma: a rare but challenging entity

Elena Trevisi  1 , Anna La Salvia  2 , Lorenzo Daniele  3 , Maria Pia Brizzi  4 , Giovanni De Rosa  3 , Giorgio V Scagliotti  4 , Massimo Di Maio  5
Affiliations
Review

Neuroendocrine breast carcinoma: a rare but challenging entity

Elena Trevisi et al. Med Oncol. .

Abstract

Breast carcinoma with neuroendocrine differentiation, also known as neuroendocrine breast carcinoma (NEBC), includes a heterogeneous group of rare tumors, which account for 2-5% of all invasive breast carcinomas. Because of their low incidence, most of the current limited knowledge of these tumors derives from anecdotal case reports or small retrospective series. The diagnosis of NEBC is based on the presence of morphological features similar to gastrointestinal and lung NETs and neuroendocrine markers. NEBCs are usually hormone receptors positive and HER2 negative, but despite this luminal phenotype, most recent studies suggested that NEBC could be associated with worse prognosis compared to invasive breast cancer without neuroendocrine differentiation. Due to its rarity and lack of randomized data, there is little evidence to guide the choice of treatment, so NEBC is currently treated as any invasive breast carcinoma not-otherwise specified. Recently, attempts to molecularly characterize NEBC have been made, in order to provide new targets for a more personalized treatment of this uncommon entity.

Keywords: Breast cancer; Neuroendocrine breast carcinoma; Neuroendocrine differentiation; Neuroendocrine tumor; Small cell breast cancer.

PubMed Disclaimer

Conflict of interest statement

ET, ALS, LD, MPB, GDR have no conflict of interest to declare. GVS received honoraria, research funding and had roles as consultant or advisor for Roche, Pfizer, AstraZeneca, Lilly Pharma and MSD. MDM received honoraria and had roles as consultant or advisor for AstraZeneca, Bristol Myers Squibb, MSD, Eisai, Takeda, Pfizer and Janssen.

Figures

Fig. 1
Fig. 1
Pathological findings in a large cells neuroendocrine carcinoma of the breast (WHO [11]). a H&E stain, ×10, b on immunohistochemistry, tumor cells show diffuse positive stain for Chromogranin A (×10), and c estrogen receptors (×10)
See this image and copyright information in PMC

References

    1. Lakhani SR, Ellis IO, Schnitt SJ, et al. WHO classification of tumours of the breast. 4. Lyon: International Agency for Research on Cancer; 2012.
    1. Feyrter F, Hartmann G. On the carcinoid growth form of the carcinoma mammae, especially the carcinoma solidum (gelatinosum) mammae. Frankf Z Pathol. 1963;73:24–39. - PubMed
    1. Cubilla AL, Woodruff JM. Primary carcinoid tumor of the breast: a report of eight patients. Am Surg Pathol. 1997;1:283–292.
    1. Tavassoli FA, Devilee P. World Health Organization Classification of tumours: pathology and genetics of tumours of the breast and female genital organs. Lyon: IARC Press; 2003. pp. 32–34.
    1. Bussolati G, Gugliotta P, Sapino A, et al. Chromogranin-reactive endocrine cells in argyrophilic carcinomas (“carcinoids”) and normal tissue of the breast. Am J Pathol. 1985;120(2):186–192. - PMC - PubMed

Substances