乳腺神经内分泌肿瘤诊治争议与进展

Controversies and research prrogress on the diagnosis and treatment of breast neur-oendocrine neoplasm

乳腺神经内分泌肿瘤是一组异质性肿瘤,既包括乳腺神经内分泌肿瘤,也包括伴有神经内分泌分化的乳腺浸润性癌。从20世纪60年代开始,学术界对此类肿瘤的定义、诊断标准等方面经历了反复的变化。由于对其缺乏严格的定义,此类肿瘤的名称较为混乱,诊断名词应用存在争议。世界卫生组织(WHO)第5版提出了新的乳腺神经内分泌肿瘤诊断标准。本文从乳腺神经内分泌肿瘤的命名沿革与定义、分类和病理形态学特点及免疫标志物、分子遗传学变化、临床特征、临床治疗和预后以及目前仍存在的问题等多个方面进行梳理。鉴于乳腺神经内分泌肿瘤仍有较多需要解决的问题,建议国内同仁制定乳腺神经内分泌肿瘤相关诊断术语的使用规范,在对临床上比较罕见的原发性乳腺神经内分泌肿瘤进行深入研究的同时,针对伴有神经内分泌分化的浸润性乳腺癌开展多中心、分层规范化研究。

Breast neuroendocrine neoplasm is a heterogeneous tumor group,which includes both breast neuroendocrine neoplasms and in-vasive breast carcinomas with neuroendocrine differentiation.Since the 1960s,the definition and diagnostic criteria of breast endocrine car-cinoma have changed repeatedly.Owing to the lack of a strict definition,the diagnostic terminology for such tumors is unclear and contro-versial.The fifth edition of the WHO classification of breast neoplasms proposes a new diagnostic criterion for breast neuroendocrine neo-plasms.This article reviews the revisions of the pathological definition and diagnostic terms,classification and pathological-morphological characteristics and immune markers,molecular genetic changes,clinical features and treatment,and prognosis of primary breast neuroen-docrine neoplasms and some existing issues.As lots of issues about primary breast neuroendocrine neoplasms remain unresolved,formulat-ing specifications is suggested for using relevant diagnostic terms;conducting multicenter,stratified standardized studies on invasive breast cancer with neuroendocrine differentiation;and conducting in-depth studies on primary breast neuroendocrine neoplasms.