伴有神经内分泌分化的乳腺梭形细胞癌

Spindle cell carcinoma of breast with neuroendocrine differentiation

目的探讨乳腺伴有神经内分泌分化的梭形细胞癌的病理形态学和免疫表型特点及鉴别诊断。方法复习2500例乳腺癌切片，找出以梭形细胞占主要优势（〉80％）的癌5例，其中2例梭形细胞型导管内癌和3例梭形细胞型浸润癌。采用HE、阿辛蓝（AB）／PAS和网织染色，以及用癌胚抗原（CEA）、上皮膜抗原（EMA）、细胞角蛋白（CK7、3413E12、AE1／AE3）、神经元特异性烯醇化酶（NSE）、突触素、嗜铬蛋白（cg）A、Lue-7、波形蛋白，S-100、平滑肌肌动蛋白（SMA）、calponin、雌激素受体（ER）、孕激素受体（PR）、c—erbB-2、E-钙黏素、Ki-67、p53抗体进行免疫组织化学观察。其中4例有随访信息。结果患者平均年龄在68岁。镜下：5例癌细胞形态主要为长梭形的上皮样细胞，3例有少数胞质内空泡状细胞，4例可见散在AB阳性细胞。免疫组织化学5例均表达AE1／AE3、EMA、CEA、E-钙黏素和突触素，CK7有4例表达，NSE阳性3例，CgA和Lue7阳性2例，ER阳性4例，PR阳性2例，1例表达c-erbB-2，1例有灶状波形蛋白阳性。免疫组织化学结果显示2例梭形细胞型导管内癌和1例梭形细胞型浸润性癌是梭形细胞型的神经内分泌癌，另外2例梭形细胞型浸润性癌是伴有神经内分泌分化的化生性癌。随访3例存活（24～58个月），1例27个月内死亡。结论上皮样梭形细胞和细胞内黏液的出现是乳腺伴有神经内分泌分化癌的一个形态学特点。梭形细胞神经内分泌型导管内癌需要和普通导管增生及导管内乳头状瘤鉴别。梭形细胞型的神经内分泌癌和伴神经内分泌分化的梭形细胞浸润性癌需要与梭形细胞肌上皮肿瘤、恶性黑色素瘤及某些软组织肿瘤鉴别。

Objective To describe the morphologie features and immunohistoeheraistry of spindle cell carcinoma of breast with neuroendoerine differentiation. Methods Retrospective review of 2500 cases of breast carcinoma showed 5 cases （ 0. 2% ） with a predominance （ 〉 80% ） of spindle cell component. Amongst the 5 cases studied, 2 represented intraduetal spindle cell carcinoma and 3 represented invasive spindle cell carcinoma. The paraffin sections were stained with hematoxylin and eosin, alcian blue, periodic aeid-Sehiff and retieulin stain. Immunohistoehemieal studies for AE1/AE3, CEA, EMA, CK7, 34[3E12, NSE, synaptophysin, ehromogranin A, Leu-7, vimentin, S-100, SMA, ealponin, estrogen receptor, progesterone receptor, e-erbB2, E-cadherin, Ki-67 and p53 were also carried out. Follow-up information was available in 4 of the 5 cases. Results The mean age of the patients was 68 years. Histologically, all tumors were predominantly composed of elongated spindle cells. Three of these cases also contained tumor cells with vaeuolated cytoplasm, aleian blue-positive tumor cells were observed in 4 cases. Immunohistoehemically, the spindle tumor cells in all cases expressed AE1/AE3, CEA, EMA, E-eadherin and synaptophysin. CK7 was positive in 4 cases, NSE in 3 cases, ehromogranin A and Leu-7 in 2 cases. Estrogen receptor was expressed in 4 eases and progesterone receptor in 2 eases. Overexpression of e-erbB2 oneoprotein was detected in only 1 case. Vimentin was focally positive in 1 case. Two cases of intraduetal spindle cell carcinoma and 1 of the 3 cases of invasive spindle cell carcinoma were classified as neuroendoerine carcinoma of spindle cell type, while the remaining 2 cases of invasive spindle cell carcinoma were considered as metaplastie carcinoma with neuroendocrine differentiation. Amongst the 4 patients with follow-up information available, 3 were still alive 24 to 58 months after the initial diagnosis. One patient died within 27 months of diagnosis. Conclusions The presence of spindle tumor cells and sometimes intracytoplasmie muein are useful morphologie clues in diagnosing spindle cell carcinoma of the breast with neuroendoerine differentiation. Intraduetal neuroendoerine spindle cell carcinoma needs to be distinguished from usual duetal hyperplasia and intraduetal papilloma. On the other hand , invasive spindle cell carcinoma with neuroendocrine differentiation needs to be distinguished from spindle cell myoepithelioma, melanoma and sometimes soft tissue neoplasm.