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:肿痛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.ONB 34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,CKpan在鳞状及腺样分化的细胞100%表达,肌红蛋白表达于横纹肌母细胞分化的细胞.未成熟型恶性畸胎瘤内可见原始神经组织,有神经管结构及大片的神经胶质细胞(GFAP阳性).未发现幼稚的非角化透明鳞状细胞巢.1例胚胎组织鼻腔、口腔可见衬覆非角化透明鳞状细胞.结论 SNTCS是罕见的高度恶性肿瘤,多数ONB为低级别的恶性肿瘤.SNTCS成分复杂,形态多样,取材不充分可能导致误诊.未发现SNTCS为生殖细胞来源肿瘤的证据,将SNTCS命名为鼻腔鼻窦畸胎样癌肉瘤可能更好,其组织发生可能为嗅/鼻腔鼻窦黏膜中的原始全能细胞,与ONB的关系有待于进一步的研究和证实.

abstractsObjective To study the clinicopathologic features, diagnosis and differential diagnosisof sinonasal teratocarcinosarcoma (SNTCS)and olfactory neuroblastoma (ONB), and to discuss thehistogenesis and possible relationship between SNTCS and ONB. Methods Seven cases of SNTCS and 34cases of ONB were retrieved from the pathological archives together with one case each of malignant teratomaand immature embryonic tissue at 8 weeks were collected from Beijing Tongren Hospital. Theclinicopathologic features were analyzed and immunohistochenncal staining was performed on paraffinsections. Results Six of the SNTCS patients were male and one was female. The patients age range was 25to 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 fromectoderm. Glandular and tubular structures and ciliated columnar epithelium derived from endoderm.Fibroblasts, striated muscle, smooth muscle, cartilage and osteoid matrix derived from mesoderm. Thecarcinoma component exhibited mostly adenocarcinoma and squamous cell carcinoma, whereas the sarcomacomponent mostly exhibited rhabdomyosarcoma, leiomyosarcoma, and fibrosarcoma. In addition, carcinoid,and primitive mesenchymal tissue and the ONB component were also seen. The morphological characteristicsof SNTCS comprised fetal clear cell squamous epithelium, carcinosarcoma and the ONB component. Byimmunohistochemistry, the epithelial component and cells with epithelium differentiation were positive forcytokeratin (pan) and EMA. The ONB component was positive for Syn, NSE, CD99, NF and CgA todifferent degrees. Neurofibril bundles were positive for S-100, and Flexner-Wintersteiner rosettes expressedcytokeratin (pan) and EMA. The spindle cells expressed vimentin, SMA, desmin, myosin and myoglobin.The primitive mesenchymal tissue expressed vimentin, and the mucoid materials and glycogen were positivefor PAS. GFAP was negative in all cases. The 34 cases of ONB, included 18 men and 16 women, the ageranged from 12 to 72 years (mean 42. 8 years). Microscopically, the tumor shows epithelial nests, net ofangioma-like fibrous connective tissues, small round and spindle cells, glandular, squamous-like cells, andcells of rhabdomyoblastic differentiation, Homer-Wright and Flexner rosette, bundles of neurofibrils, etc.NSE and CgA were expressed in small cells. S-100 protein was positive in the areas of bunches ofneurofibril. Cytokeratin (pan) was positive in epithelial cells. Myoglobin was positive in the cells ofrhabdomyoblastic differentiation. The single case of immature malignant teratoma exhibited primitive nervetissue, but fetal clear cell squamous epithelium was not found. In the immature embryonic tissue,rudimentary organs were formed, with fetal clear cell squamous epithelium lining present on the nasal andoral cavities surface. Conclusions SNTCS is a rare and aggressive malignant neoplasm. Most of ONB arelow-grade malignant tumors. Morphological differences are the most important basis to make differentiateSNTCS from ONB. As SNTCS may demonstrate a multiplicity of structures and pleomorphism, inadequatesampling at biopsy, therefore, may lead to errors in diagnosis. No evidence show that SNTCS are derivedfrom germ cells and sinonasal teratoid carcinosarcoma may be a more proper name. SNTCS probably arisesfrom primitive totipotential cells of olfactory/sinonasal membrane, and the relationship between SNTCS andONB needs further study.