Neuroendokrine Neoplasien der Mamma

Abstract

Neuroendocrine neoplasms (NEN) of the breast are specific tumor entities. According to the literature up to 5% of breast neoplasms are malignant epithelial neoplasms of the breast. They are defined by a neuroendocrine (NE) architecture and cytology combined with an expression of the neuroendocrine vesicle markers chromogranin A and/or synaptophysin. The diagnosis is supplemented by the receptor status and the proliferative activity. According to the World Health Organization (WHO) classification of 2012 the following groups of NEN are distinguished: (1) invasive breast carcinoma with NE differentiation, (2) well-differentiated neuroendocrine tumor (NET) and (3) poorly differentiated small cell carcinoma (NEC). This review article focuses on (1) the definition and basic principles of diagnostics, (2) the history, nomenclature and WHO classification from 2003 and 2012, (3) the frequency of breast NEN, (4) the hereditary background and functional activity, (5) the expression of receptors and (6) the possible clinical implications. In addition, the first results of a retrospective single center study (n = 465 patients with breast cancer over a time period of 4 years) on the frequency of NEN of the breast at the Breast Center of the University Hospital Düsseldorf are presented. In this study a frequency of 4.5% of NEN was found based on a diagnostic cut-off of > 50% Chromogranin A and/or synaptophysin positive tumor cells.

Primary Neuroendocrine Breast Carcinoma in a 13-Year-Old Girl: Ultrasonography and Pathology Findings

“…Mammary localization is very rare, accounting for less than 0.1% of all breast cancers and less than 1% of neuroendocrine tumors [1, 5]. Our observation illustrates the diagnostic difficulties encountered in practice by underequipped pathological laboratories in Black Africa (e.g., absence of immunodetection technology and electron microscopy).…”

“…Histologically, the breast NEC is characterized by cell proliferation appearing as pseudorosettes invading the surrounding adipose tissue, with a richly vascularized small or fibrous stroma and small monomorphic cell elements with rather irregular nuclei and weak mitotic activity [1, 16]. Depending on the cell type, grade, degree of differentiation, and presence of mucin production, several subtypes are defined in the WHO classification.…”

“…Neuroendocrine carcinomas (NECs) constitute a very rare entity, affecting mainly the bronchopulmonary system and gastrointestinal tract [1, 2]. Breast localizations are unusual and represent less than 0.1% of mammary cancers and less than 1% of neuroendocrine tumors [1].…”

“…Breast localizations are unusual and represent less than 0.1% of mammary cancers and less than 1% of neuroendocrine tumors [1]. Most publications in radiology describe nonspecific suspicious findings and do not indicate consistent imaging characteristics of this particular carcinoma by all the available modalities, including ultrasonography, mammography, and MRI [3].…”

Triple receptor-positive primary neuroendocrine carcinoma of breast in a young patient

“…Tumours which overexpress somatostatin receptors are amenable to be treated by long-acting somatostatin analogues (SSAs). Advanced metastatic and/or inoperable patients with BCNF may be treated with peptide receptor radionuclide therapy with radiolabelled SSA 24. The disease is most commonly described in the age group ranging from 40 to 70 years, more frequently in women aged above 60 years 3.…”