Neuroendocrine carcinoma of the breast is considered a rare entity, and for this reason there are no data from prospective clinical trials on its optimal management. Early stage tumors are usually treated with the same strategy used for the other types of invasive breast cancer. Anthracycline-and taxane-based regimens represent the most frequently administered chemotherapy in neoadjuvant and adjuvant setting, as well as for metastatic disease, although combinations of platinum compounds and etoposide have been widely used, in particular for small-cell histology and tumors with a high proliferation index. For metastatic disease, a multimodality therapeutic strategy can be considered on an individual basis, with chemotherapy, endocrine therapy, peptide receptor radionuclide therapy, radiation therapy, surgery, or a combination of the above. In the near future, a better knowledge of the biology of these tumors will hopefully provide new therapeutic targets for personalized treatment. In this review, we discuss the current evidence and the future perspectives on diagnosis and treatment of neuroendocrine carcinoma of the breast. The Oncologist 2016;21:28-32 Implications for Practice: Neuroendocrine carcinoma of the breast (NECB) is a distinct entity of breast cancer. Clinical features and morphology are not helpful to distinguish NECB from other subtypes of breast cancer; therefore, immunohistochemistry markers for neuroendocrine differentiation, mainly chromogranin and synaptophysin, should be routinely used to confirm the diagnosis, especially in cases of mucinous or solid papillary carcinoma in which the suspicion of NECB may be relevant. Adjuvant treatment should be offered according to the same recommendations given for the other types of invasive breast cancer. An accurate diagnosis of NECB is also important in the metastatic setting, in which a multimodality approach including specific therapies such as peptide receptor radionuclide therapy can be considered.

As patients with previous history of pancreatitis were excluded from the study, CT findings allowed correct characterization of only 60% of cystic pancreatic masses. Among the remaining 40%, 15-20% of the wrong diagnoses could not be corrected by means of CT, given the patterns shown by the tumors. In 20-25% of the cases, a nonspecific diagnosis of cystic mass was made.

Intraductal papillary mucinous tumor (IPMT) of the pancreas was identified and classified only recently. IPMT has a primarily intraductal, papillomatous growth pattern, which is associated with excessive mucin secretion and results in progressive ductal dilatation or cyst formation. The tumor occurs in four forms: segmental or diffuse involvement of the main pancreatic duct and macrocystic or microcystic involvement of a branch duct. In the past, many IPMTs may have been misdiagnosed as chronic pancreatitis because of their generally benign behavior. The correct diagnosis, once achieved only with endoscopic retrograde cholangiopancreatography (ERCP), can now be made with noninvasive imaging modalities, particularly computed tomography (CT) and magnetic resonance (MR) imaging. ERCP remains the imaging modality of choice for diagnosis of IPMT. With ERCP, the communication between the cystically dilated ductal segment or branch duct and the main pancreatic duct is easily demonstrated. However, reflux of contrast material due to an excess of mucin or an enlarged papillary orifice hinders filling of the ductal tree. Filling defects due to mucin globs or mural nodules are also important clues to the diagnosis. Bulging of the papilla into the duodenal lumen is virtually pathognomonic of IPMT and is well demonstrated with CT or MR imaging.