Primary neuroendocrine neoplasm of the breast (BNEN) is an uncommon breast neoplasm, and in most cases, it presents as hormone receptors positive and HER-2 negative. Moreover, in neuroendocrine neoplasms (NENs), the signet ring feature is a rare morphological subtype, and only a few cases have been reported. Here, we report the case of a primary breast neuroendocrine neoplasm with an unusual signet ring cell appearance in this paper. The documentation of this case, combined with a review of the literature, may add to existing knowledge about the outcome and management of this rare tumor.
In the present review, we describe a unique case of HER-2-positive primary BNEN with a signet ring feature that has not been reported in English. Additionally, we performed a literature search of the PubMed and Web of Science databases and calculated statistics for clinical data and follow-up.
Our literature search, excluding non-English literature, identified 15 articles with data from 24 cases, including ours. The mean age was 51.25 years (range, 30–79 years), and there were 13 male patients (54%) and 11 female patients (46%). Of the 24 cases, some cases (11/24) were associated with lymph node metastases, a few cases (6/24) had distant metastasis, and the vast majority of cases (23/24) occurred in the digestive system. Primary hepatic signet ring cell neuroendocrine tumor showed slow progression and good prognosis. Lymph node involvement was identified in one of eight (12.5%) documented cases, and one of eight (12.5%) reported cases presented with distant metastatic disease. However, the prognosis of neuroendocrine tumors with signet ring cells in the pancreas and stomach was poor. Lymph node involvement was identified in 9 of 15 (60%) documented cases, and 5 of 15 (33.3%) reported cases presented with distant metastatic disease.
NENs with a signet ring feature is uncommon, and this is the first case report of its occurrence in the breast. Current knowledge is limited to anecdotal experience based on case reports and small case series. We provide a literature review to summarize knowledge about this rare entity.