Le neoplasie neuroendocrine della mammella: inquadramento diagnostico e gestione clinica

Breast Neuroendocrine Neoplasia (or Breast NENs, BNENs) represent the minority of breast neoplasia (about 0.5–1% of all breast cancers) and, generally, one of the rare sites of origin within NENs. Although the morphology of these forms may suggests their possible neuroendocrine origin, the diagnosis of BNEN is based on the positivity of neuroendocrine markers. Their clinical management appears to be a controversial area to date, and the therapeutic strategy of BNENs often turns out to be overlapping with that of invasive breast carcinomas, thus providing surgery as an initial approach if feasible/indicated, followed by systemic treatment with adjuvant and/or palliative intent in case of advanced disease, with different therapeutic options, from chemotherapy to hormone therapy, including also anti-HER2 and possible target treatments. Future studies will be needed to be able to enable validation of the optimal therapeutic strategy for BNENs and, at the same time, to lead to personalisation of treatment of this rare subgroup of neuroendocrine neoplasms.