目的:探讨乳腺囊内乳头状癌(intracystic papillary carcinoma,IPC)伴神经内分泌分化的临床病理学特征及诊断和鉴别诊断.方法:分析1例乳腺囊内乳头状癌伴神经内分泌分化的临床和病理学改变,结合文献对该肿瘤的临床表现、病理形态学特征、诊断和鉴别诊断要点进行讨论.结果:囊内乳头状癌大体呈结节状,切面呈囊性,囊内壁见乳头状突起,暗红色.镜下肿瘤大部分区域呈实性,部分区域呈乳头状,乳头中央为纤细的纤维脉管性轴心,癌细胞复层排列,呈短梭形或卵圆形,未见核分裂像;灶区可见癌细胞在囊壁内微浸润.免疫组化显示囊内肿瘤细胞弥漫强阳性表达ER、PR,局灶性或弱阳性表达C-erbB-2、34βE12、NSE、Syn和CD57,而不表达 SMA、p63、CD10、S-100蛋白、CgA,Ki-67增殖指数约5%.结论:IPC伴神经内分泌分化是乳腺十分少见的上皮性肿瘤,由于组织学形态多样,同时具有两种肿瘤的特征,常易引起病理误诊,多数IPC和神经内分泌乳腺癌患者预后较好.%Objective :To explore the pathologic characteristics and differential diagnosis of intracystic papillary carcinoma ( IPC )of female breast with neuro-endocrine differentiation of the breast. Methods: One case of IPC with neuro-endocrine differentiation was examined by clinicopathologic changes, with reviewing the literatures involved in clinical demonstrations, pathological features, diagnosis and differential diagnosis.Results: Grossly, the tumor presented itself with irregular nodule, grayred colored and intracystic section, fibrotic cyst wall and projection into cystic space. Microscopy showed solid and papillary area inside the capsule wall and that the papilla contained fibrovascular stalks and had more layers of malignant cells which were orbicular-ovate or of shuttle form, but free of mitotic figures detected. Minor papillary proliferation was also seen in the lesion. Immunohistochemisty revealed diffusely strong positive ER and PR for the tumor cells and focal or weak positive C-erbB-2,34βE12,NSE, Syn and CD57. Yet, SMA, p63, CD10, S-100 and CgA were found negatively. Ki-67 indicated that the proliferation index of the tumor cells was about 5％. Conclusion: IPC with neuro-endocrine differentiation is a rare epithelial tumor, which otherwise leads to misdiagnosis due to its diverse histology but common feathers of the two tumors in morphology. Prognosis seems to be good for majority of the patients with IPC or neuroendocrine breast carcinoma.
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