鼻腔鼻窦畸胎癌肉瘤与嗅神经母细胞瘤的对比观察  

Clinicopathologic study of sinonasal teratocarcinosarcoma and its contrast with olfactory neuroblastoma

作　　者：李雪[1] 刘红刚[1] 谢新纪[1] 韩一丁[1] 李明[1] 

机构地区：[1]首都医科大学附属北京同仁医院病理科,100730

出　　处：《中华病理学杂志》2008年第7期458-464,共7页Chinese Journal of Pathology

摘　　要：目的总结鼻腔鼻窦畸胎癌肉瘤（SNTCS）的临床病理特征、诊断和鉴别诊断要点以及嗅神经母细胞瘤（ONB）的临床病理特点，探讨SNTCS与ONB的关系及其组织来源。方法对7例SNTCS和34例ONB的临床病理情况及免疫表型进行了观察，并对照观察了1例未成熟型恶性畸胎瘤及1例妊娠8周胚胎组织的形态学及免疫表型特点。结果SNTCS男6例，女1例，平均年龄46岁。7例中3例为复发病例。组织病理学特征：肿瘤由多种组织成分构成，这些成分源自3个胚层，是畸胎瘤样成分和癌肉瘤的混合，畸胎瘤样成分包括外胚层幼稚的鳞状细胞巢，内胚层的腺体和管状结构、纤毛柱状上皮，中胚层的纤维细胞、软骨、骨样基质、横纹肌及平滑肌组织；癌成分主要包括腺癌、鳞状细胞癌成分，肉瘤成分主要为横纹肌肉瘤、平滑肌肉瘤和纤维肉瘤成分，另外可见ONB成分、类癌及原始间叶组织。免疫组织化学染色：上皮及向上皮分化的组织表达广谱细胞角蛋白（CKpan）及上皮细胞膜抗原（EMA）；ONB成分不同程度地表达突触素（Syn）、神经元特异性烯醇化酶（NSE）、CD99、神经丝蛋白（NF）及嗜铬粒素A（CgA），S-100蛋白神经丝束阳性。真菊形团表达CKpan及EMA；梭形细胞成分表达波形蛋白、平滑肌肌动蛋白（SMA）、结蛋白、肌球蛋白、肌红蛋白。原始间叶组织表达波形蛋白。黏液样物质和糖原颗粒PAS阳性。7例肿瘤细胞胶质纤维酸性蛋白（GFAP）均为阴性。ONB34例，男18例，女16例，平均年龄42．8岁。形态学特征是：分叶状上皮团巢，血管襻网隔，小圆小梭形细胞，异向分化的腺样、鳞状上皮样细胞及横纹肌母细胞，菊形团，神经丝束，深染的细胞核，少、粉染或透明的胞质。免疫组织化学染色：NSE及CgA在小细胞100％表达，但在不同病例表达程度不同，S-100蛋白在神经丝处100％表达Objective To study the clinicopathologic features, diagnosis and differential diagnosis of sinonasal teratocarcinosarcoma （SNTCS）and olfactory neuroblastoma （ONB）, and to discuss the histogenesis and possible relationship between SNTCS and ONB. Methods Seven cases of SNTCS and 34 cases of ONB were retrieved from the pathological archives together with one case each of malignant teratoma and immature embryonic tissue at 8 weeks were collected from Beijing Tongren Hospital. The clinicopathologic features were analyzed and immunohistochemical staining was performed on paraffin sections. Results Six of the SNTCS patients were male and one was female. The patients age range was 25 to 69 years （ mean age 46）. Four cases were initial presentation and three were recurrences. Histologically, the tumor shows multiple tissue components derived from three germ layers. There were mixture of teratoma-like tissue and carcinosarcoma. The components include fetal clear cell squamous epithelium derived from ectoderm. Glandular and tubular structures and ciliated columnar epithelium derived from endoderm. Fibroblasts, striated muscle, smooth muscle, cartilage and osteoid matrix derived from mesoderm. The carcinoma component exhibited mostly adenocarcinoma and squamous cell carcinoma, whereas the sarcoma component mostly exhibited rhabdomyosareoma, leiomyosarcoma, and fibrosareoma. In addition, carcinoid, and primitive mesenchymal tissue and the ONB component were also seen. The morphological characteristics of SNTCS comprised fetal clear cell squamous epithelium, carcinosarcoma and the ONB component. By immunohistochemistry, the epithelial component and cells with epithelium differentiation were positive for cytokeratin （pan） and EMA. The ONB component was positive for Syn, NSE, CD99, NF and CgA to different degrees. Neurofibril bundles were positive for S-100, and Flexner-Wintersteiner rosettes expressed eytokeratin （pan） and EMA. The spindle cells expressed vimentin, SMA, desmin, myosin and myoglobin. The prim

关 键 词：癌肉瘤 鼻窦肿瘤 成感觉神经细胞瘤 嗅觉 

分 类 号：R686[医药卫生—骨科学]