乳腺原发神经内分泌癌临床病理特征分析

Clinicopathologic Characteristics of Primary Neuroendocrine Carcinoma of the Breast

目的:探讨乳腺原发神经内分泌癌(NECB)的临床病理特征、诊断与鉴别诊断及预后。方法:收集7例乳腺原发神经内分泌癌病例,观察其临床表现、组织病理学及相关免疫组化表型,收集随访资料并复习相关文献。结果:患者均为女性,年龄32-82岁之间。肿物位于左乳5例,右乳2例。临床均表现为可触及性包块部分伴有疼痛,其中4例行乳腺改良根治术,2例行乳腺单纯切除术,1例行肿物切除术。巨检见肿物直径1-4cm不等,切面灰黄、灰红实性,质软,膨胀性生长。镜下见肿瘤细胞呈梭形或透明细胞样,呈实性巢状、腺泡样排列,间质见纤维及血管分隔,其中2例可见黏液产生。肿瘤细胞免疫组织结果CgA、Syn、NSE和CD56阳性。随访5-68个月,均未发生肿瘤进展。结论:NECB罕见,诊断需依靠临床及镜下组织学特征,神经内分泌免疫组织化学标记在诊断及鉴别诊断上具有重要价值。

Objective:It aims to investigate clinicopathologic characteristics and diagnosis of primary neurendocrine carcinoma of the breast(NECB).Methods:The clinical,histopathological and immunohistochemical characteristics of 7 patients had primary NECB were analyzed.The clinical and follow-up information was obtained and the relevant literatures were reviewed.Results:The patients were females that aged between 32 and 82 years old.There were 5 patients had tumor in the left breast while 2 patients had it in right breast.The clinical manifestations were breast lump with partial pain.Among them,4 patients had modified eradicative operation of breast,2 patients had simple excision of breast,and 1 patient had lump resection.Generally,all tumor lumps had 1-4 cm in diameter with grayish yellow/red section,soft texture and expansive growth.Moreover,the tumor cells looked like fusiform or clear cells.They were solid nested and separated by interstitial fibers and blood vessels.In addition,mucus was found in 2 cases.The immunohistochemistry showed that neurendocrine carcinoma cells were positive for CgA,Syn,NSE and CD56.After follow-up for 5-68 months,no tumor progression was found.Conclusion:NECB is rare tumor,and it should be definitely diagnosed by integration of clinical and pathological features.Furthermore,chemical marking of neuroendocrine immune tissue has important value on diagnosis of NECB.