Salemis Nikolaos S
Breast Cancer Surgery Unit, Army General Hospital, Athens, Greece.
IASO Women's Hospital, Athens, Greece.
Intractable Rare Dis Res. 2020 Nov;9(4):233-246. doi: 10.5582/irdr.2020.03046.
Primary neuroendocrine carcinoma of the breast (NEBC) is a very rare occurrence accounting for less than 0.1% of all breast cancers. Typically, the tumor presents with ER- and PgR-positive and HER-2-negative status. Despite its luminal type, NEBC is associated with a more aggressive clinical course and poorer prognosis compared to the other types of invasive breast cancer. Clinical and radiological findings are nonspecific. The most common clinical manifestation is a palpable mass whereas in mammography the tumor most commonly appears as a round or oval mass without spiculated margins. Herein, a very rare case of NEBC is described in an asymptomatic patient who presented with an area of architectural distortion and the presence of microcalcifications that was incidentally detected on a screening mammography. A review of the literature has also been conducted. The diagnosis of NEBC requires a thorough investigation to exclude the possibility of a metastatic neuroendocrine tumor from another site because the two entities require different treatment approaches. Due to the rarity of the disease, the optimal therapeutic approach has not been clearly defined. Surgical resection is the mainstay of treatment. Further research is needed to better understand the molecular characteristics of NEBC and identify novel targeted therapies.
乳腺原发性神经内分泌癌（NEBC）极为罕见，占所有乳腺癌的比例不到0.1%。通常，该肿瘤表现为雌激素受体（ER）和孕激素受体（PgR）阳性、人表皮生长因子受体2（HER-2）阴性。尽管NEBC属于管腔型，但与其他类型的浸润性乳腺癌相比，其临床病程更具侵袭性，预后更差。临床和影像学表现不具有特异性。最常见的临床表现是可触及的肿块，而在乳腺钼靶检查中，肿瘤最常表现为圆形或椭圆形肿块，边缘无毛刺。本文描述了一例非常罕见的NEBC病例，患者无症状，在筛查乳腺钼靶检查时偶然发现有结构扭曲区域并伴有微钙化。同时也对相关文献进行了综述。NEBC的诊断需要进行全面检查，以排除其他部位转移性神经内分泌肿瘤的可能性，因为这两种实体需要不同的治疗方法。由于该疾病罕见，最佳治疗方法尚未明确界定。手术切除是主要治疗手段。需要进一步研究以更好地了解NEBC的分子特征并确定新的靶向治疗方法。
