Neuroendocrine breast tumors:clinicopathological analysis of 6 cases
张荣辉 1魏潇 1李思 1黄晓娟 1袁静萍2
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作者信息
1. 武汉市武昌医院病理科,武汉 430000
2. 武汉大学人民医院病理科,武汉 430060
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摘要
目的 探讨乳腺神经内分泌肿瘤(neuroendocrine neoplasms of the breast,NENB)的临床病理特征、诊断及鉴别诊断,查找相关文献对该疾病进行总结复习.方法 回顾性研究武汉市武昌医院和武汉大学人民医院2016-2022年诊断为NENB的病例,所有病例均由2位高年资病理医师复阅.结果 6例患者,年龄36～65岁,肿瘤最大径平均2.3 cm,均以单侧乳腺肿块为首发症状(6/6),其中3例(3/6)发生在左侧,3例(3/6)发生在右侧;3例(3/6)患者行乳腺癌改良根治术,3例(3/6)行保乳术,其中1例保乳术患者术后2年复发,再次手术行根治术.病理分型:乳腺神经内分泌瘤(neuroendocrine tumor of the breast,NETB)3例,小细胞癌(small cell neuroendocrine carcinoma,SCNECC)2例,乳腺混合性神经内分泌癌(breast mixed neuroendocrine carcinoma,Br-MiNEN)1例.6例NENB免疫表型为:Syn和/或CgA弥漫阳性,其中3例NETB的ER/PR均为阳性,HER2均阴性,2例SCNECC的ER/PR阴性、HER2均阴性,1例Br-MiNEN的ER/PR阳性、HER2阴性.Syn和/或CgA弥漫阳性是诊断NENB特异性的标志物.术后随访时间2年至8年,5例患者(5/6)随访无病生存,1例Br-MiNEN患者(1/6)失访.结论 NENB十分罕见,临床表现及影像学不具有特征性,典型的神经内分泌化的组织学形态和免疫表型有助于其诊断及鉴别诊断.
Abstract
Objective To explore the clinical and pathological characteristics,diagnosis,and differential diagnosis of neu-roendocrine neoplasms of the breast (NENB),and to review relevant literature on the disease.Methods A retrospective study was conducted on cases diagnosed as NENB at Wuhan Wuchang Hospital and Wuhan University People's Hospital from 2016 to 2022.All cases were reviewed by two senior pathologists.Results Six patients,aged 36 to 65 years,with an average maximum tumor diameter of 2.3 cm,all presented with a unilateral breast mass as the first symptom (6/6).Three cases (3/6) occurred on the left side,and three cases (3/6) occurred on the right side.Three patients (3/6) underwent modified radical mastectomy,and three patients (3/6) underwent breast-conserving surgery.Among the breast-conserving surgery patients,one had a recurrence 2 years after surgery and underwent a second surgery for radical resection.Pathological subtypes included:neuroendocrine tumor of the breast (NETB) in 3 cases,small cell neuroendocrine carcinoma (SCNECC) in 2 cases,and breast mixed neuroendocrine carcinoma (Br-MiNEN) in 1 case.The immuno-phenotype of the 6 NENB cases was as follows:Syn and/or CgA diffusely positive.Three NETB cases had positive ER/PR and negative HER2,two SCNECC cases had negative ER/PR and negative HER2,and one Br-MiNEN case had positive ER/PR and negative HER2.Syn and/or CgA diffuse positivity is a specific marker for diagnosing NENB.The postoperative follow-up time ranged from 2 to 8 years.Five patients (5/6) were disease-free at the time of follow-up,while one Br-MiNEN patient (1/6) was lost to follow-up.Con-clusion NENB is extremely rare,and its clinical presentation and imaging are not characteristic.Typical neuroendocrine histological morphology and immunophenotype are helpful for its diagnosis and differential diagnosis.
关键词
乳腺神经内分泌肿瘤/乳腺神经内分泌瘤/乳腺神经内分泌癌
Key words
Neuroendocrine neoplasms of the breast/breast neuroendocrine tumor/breast neuroendocrine cancer
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