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Neuroendocrine neoplasms of the breast are rare breast cancers whose cells make hormone-like granules, ranging from slow-growing tumours to small cell carcinoma like that of the lung. They are easily mistaken for ordinary breast cancer or for spread from elsewhere; slow-growing forms are treated like hormone-driven breast cancer and small cell forms with the lung small cell regimens.
The 2019 WHO classification divides neuroendocrine neoplasms of the breast into well-differentiated neuroendocrine tumour, neuroendocrine carcinoma (small cell and large cell) and, separately, invasive carcinomas of other types with neuroendocrine differentiation such as solid papillary and hypercellular mucinous carcinoma (Tan 2020). The entity as first defined by the WHO in 2012 spans well-differentiated tumours to highly aggressive small cell carcinomas; correct diagnosis needs an interdisciplinary approach because the tumours are misclassified as carcinoma with neuroendocrine differentiation, no special type carcinoma or a metastasis to the breast, and in one specialised unit only five of 612 neuroendocrine patients had a breast primary (Cancers 2020). Primary small cell neuroendocrine carcinoma of the breast is histologically indistinguishable from the lung tumour, so a primary elsewhere must be excluded, and there is no standard approach to treatment because only a limited number of cases have been reported (Int J Surg Case Rep 2017).
How it differs from its parent: the well-differentiated tumours are usually hormone-receptor positive and behave like luminal breast cancer, whereas the small cell carcinomas are treated with the platinum-etoposide regimens of small cell lung cancer rather than the breast pathways; neither has a trial of its own.
How common: no incidence figure is given in the sources read.
Treatment: well-differentiated tumours as HR-positive breast cancer on the parent page; small cell and large cell neuroendocrine carcinoma with surgery and the extrapulmonary neuroendocrine carcinoma pathway (platinum and etoposide, radiotherapy), as the case literature describes (Int J Surg Case Rep 2017).
Rare and probably under-diagnosed: five patients among 612 seen over 2008 to 2019 in one specialised neuroendocrine unit (Cancers 2020). No population figure is given in the sources read.
Most cancers start in the ducts and drain first to the axillary nodes, which is why the armpit is checked and a sentinel node is sampled.
Same organ: Triple-negative breast cancer (TNBC), Breast cancer (all types), HR-positive / HER2-negative breast cancer, HER2-positive breast cancer, Male breast cancer, Ductal carcinoma in situ (DCIS), High-risk early HR-positive breast cancer, HR-positive metastatic breast cancer after CDK4/6 inhibitors, HER2-low and HER2-ultralow metastatic breast cancer, Early HER2-positive breast cancer, HER2-positive breast cancer with brain metastases, Early triple-negative breast cancer, Metastatic triple-negative breast cancer, Basal-like 1 triple-negative breast cancer (BL1), Basal-like 2 triple-negative breast cancer (BL2), Mesenchymal triple-negative breast cancer (M), Mesenchymal stem-like triple-negative breast cancer (MSL), Luminal androgen receptor triple-negative breast cancer (LAR), Immunomodulatory triple-negative breast cancer (IM), Metaplastic breast carcinoma, Carcinoma with medullary pattern (medullary breast cancer), Adenoid cystic carcinoma of the breast, Apocrine carcinoma of the breast, Secretory carcinoma of the breast, BRCA-associated triple-negative breast cancer, Inflammatory breast cancer, Paget disease of the nipple, Phyllodes tumour of the breast, Invasive lobular carcinoma of the breast, Invasive breast carcinoma of no special type (invasive ductal carcinoma), Tubular carcinoma of the breast, Mucinous carcinoma of the breast, Papillary carcinomas of the breast (encapsulated, solid and invasive papillary), Invasive cribriform carcinoma of the breast, Invasive breast carcinoma with medullary pattern (medullary carcinoma), Invasive micropapillary carcinoma of the breast, Lobular carcinoma in situ (LCIS)
Treated as HR-positive breast cancer.
Surgery and the extrapulmonary neuroendocrine carcinoma pathway (platinum and etoposide, radiotherapy); no standard exists.
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Query for this cancer: (TITLE:"Neuroendocrine neoplasms of the breast" OR ABSTRACT:"Neuroendocrine neoplasms of the breast" OR TITLE:"Neuroendocrine carcinoma of the breast" OR ABSTRACT:"Neuroendocrine carcinoma of the breast" OR TITLE:"Neuroendocrine tumour of the breast" OR ABSTRACT:"Neuroendocrine tumour of the breast" OR TITLE:"Primary small cell neuroendocrine carcinoma of the breast" OR ABSTRACT:"Primary small cell neuroendocrine carcinoma of the breast" OR TITLE:"Breast carcinoma with neuroendocrine differentiation" OR ABSTRACT:"Breast carcinoma with neuroendocrine differentiation") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Neuroendocrine neoplasms of the breast, not a curated reading list.
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Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
Dose reduce or avoid for CrCl below 60 (carboplatin is the alternative).
Reduce to 75% for CrCl 15-50.
See all on the product pages:CisplatinEtoposide·Printable cards in the navigator
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