Neuroendocrine Tumors of the Breast

A small but increasingly recognized and studied subset of breast carcinomas are characterized by neuroendocrine (NE) differentiation. As with nearly all forms of breast neoplasia, NE tumors are characterized by considerable heterogeneity in microscopic appearance and clinical aggressiveness. About half of NE breast carcinomas recapitulate the histological spectrum typical of their counterparts in other organ systems, varying from “carcinoid-like” to small cell carcinoma, with most representing intermediate grade tumors. Despite NE morphology, these tumors have a high frequency of estrogen receptor expression. Clinical outcomes of women with NE breast carcinomas are reliably grade and stage dependent. Tumors associated with “solid papillary” differentiation comprise the remaining cases of NE breast neoplasia. Solid papillary carcinoma is an intrinsically low grade/favorable prognosis class of breast neoplasia that usually presents in post-menopausal age groups. About half of solid papillary carcinoma present as a distinctive pattern of ductal carcinoma in situ that may be difficult to recognize owing to its close resemblance to florid proliferative lesions. Invasive solid papillary carcinomas are characterized by a variety of histological patterns and often show mucinous differentiation. Future studies are necessary to better define the histogenesis, optimal classification, and improved directed therapies for NE breast neoplasia.