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形态学不典型的化生型胸腺瘤1例并文献复习OACSTPCD

Metaplastic thymoma with atypical histopathology:A case report and literature review

中文摘要英文摘要

化生型胸腺瘤(metaplastic thymoma,MT)是一类罕见类型的胸腺瘤,目前国内外多以小规模研究和个案报道对经典组织学形态的MT进行总结.现报道1例形态学不典型的MT,患者,男,44岁,因"体检发现纵隔肿物1月余"入院.影像学提示纵隔占位,患者于2022年10月在浙江大学医学院附属第一医院行"纵隔肿物切除术",大体观:灰黄脂肪样组织一块,切面见一灰白肿物,质稍硬,界限尚清.镜检示:低倍镜下肿瘤呈结节状,无明显纤维包膜,未见分叶状结构,血管周围间隙不明显,区域可见少量胶原纤维分隔;高倍镜下深染区由丰富的上皮样细胞和少许胖梭形细胞组成,背景中仅见少许淋巴细胞,部分瘤细胞呈漩涡状结构伴鳞状分化.免疫组织化学染色示:P40、P63、细胞角蛋白19(cytokeratin 19,CK19)和广谱角蛋白(cytokeratin-pan,CK-pan)阳性,黏蛋白-1(mucin-1,MUC-1)和波形蛋白(vimentin)少量阳性,CD3、CD5少量T淋巴细胞阳性,P53部分弱至中等阳性,β-联蛋白膜阳性,CD20、CD117、配对框8(paired box protein 8,PAX-8)、嗜铬粒蛋白A(chromogranin A,CgA)、突触素(synapsin,Syn)、末端脱氧核苷酸转移酶(terminal transferase,TdT)、信号转导及转录激活因子(signal transducer and activator of transcription,STAT)6和睾丸核蛋白(nuclear protein of the testis,NUT)均阴性,细胞增殖指数Ki-67低表达.YES关联蛋白1-主导控制样转录激活蛋白2(Yes associated protein 1-mastermind like transcriptional coactivator 2,YAP1-MAML2)融合基因荧光原位杂交检测结果为阳性.术后病理诊断为MT.Masaoka分期为Ⅱ期.患者术后予6次放射治疗,随访14个月为无病生存状态.

Metaplastic thymoma(MT)is a rare type of thymoma.Currently,most studies on MT,both domestically and internationally,are based on small-scale research and case reports summarizing the classical histological features of MT.Here,we report a case of morphologically atypical MT.The patient,a 44-year-old male,was admitted due to the discovery of a mediastinal mass during a health examination one month prior.A computed tomography scan showed a mediastinal mass in his chest.The patient underwent mediastinal mass resection at the First Affiliated Hospital of Zhejiang University School of Medicine in October 2022.Gross examination showed a piece of gray-yellow,adipose-like tissue with a slightly hard,gray-white mass,with relatively clear boundaries.Microscopic examination showed a nodular tumor without a distinct fibrous capsule or lobulated structure.Perivascular spaces were not prominent,with some areas showing a few collagen fibers.At high magnification,densely stained areas consisted of abundant epithelial-like cells and a few plump spindle cells,with only a few lymphocytes in the background.Some tumor cells exhibited a whorled structure with squamous differentiation.Immunohistochemistry showed positivity for P40,P63,cytokeratin 19(CK19),and cytokeratin-pan(CK-pan),with limited positivity for mucin-1(MUC-1)and vimentin.A few T lymphocytes were positive for CD3 and CD5,with partial weak to moderate positivity for P53 and membrane positivity for β-catenin.CD20,CD117,paired box protein-8(PAX-8),chromogranin A(CgA),synapsin(Syn),terminal transferase(TdT),signal transducer and activator of transcription 6(STAT6),and nuclear protein of the testis(NUT)were all negative,with a low Ki-67 proliferation index.Fluorescence in situ hybridization revealed a positive result for the Yes associated protein 1-mastermind like transcriptional coactivator 2(YAP1-MAML2)fusion gene.Postoperative pathological diagnosis confirmed MT,Masaoka stage II.The patient received six sessions of radiotherapy postoperatively and was disease-free after 14 months of follow-up.

王金文;孔梅

浙江大学医学院附属第一医院病理科,杭州 310003

化生型胸腺瘤病理特征YAP1-MAML2基因

metaplastic thymomapathological featuresYAP1-MAML2 gene

《临床与病理杂志》 2024 (003)

483-487 / 5

10.11817/j.issn.2095-6959.2024.240009

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