目的探讨鼻腔鼻窦畸胎癌肉瘤的临床病理学特征及诊断和鉴别诊断。方法对5例经病理证实的鼻腔鼻窦畸胎癌肉瘤的临床、放射影像学和病理学资料作回顾性分析,对标本行免疫组织化学染色(EnVision 法),并对所有病例进行了随访。结果患者5例,均为男性,年龄34～43岁,平均39岁。临床表现主要为单侧鼻腔堵塞、涕中带血和头痛,检查发现鼻腔充满新生物,表面光滑,有蒂,触之易出血;放射影像学均为局部占位,密度均匀,未见明显的囊性变、钙质沉着和骨化。病理检查:肿瘤多为碎组织,质地中等偏嫩,镜下观察肿瘤常由3个胚层的组织构成,外胚层的鳞状上皮团、中胚层瘤样增生的平滑肌纤维、软骨和骨组织及内胚层的富有黏液细胞的纤毛柱状上皮及排列成腺管样结构的消化道、呼吸道上皮,其中未成熟的神经上皮细胞、胞质透明的鳞状细胞团、梭形细胞肉瘤和腺癌成分最具诊断价值。免疫组织化学染色,不同胚层的组织对应相应的免疫标记,细胞角蛋白、上皮细胞膜抗原标记外胚层的复层鳞状上皮、内胚层的消化道和呼吸道上皮,波形蛋白、平滑肌肌动蛋白等标记梭形细胞肉瘤区域和平滑肌等组织,S-100蛋白、神经元特异性烯醇化酶、突触素等标记未成熟的神经上皮组织,且 nestin 阳性,CD99标记较为原始小细胞。5例患者首次病理诊断分别为毛细血管瘤、嗅神经上皮瘤、嗅神经母细胞瘤、颅咽管瘤及恶性混合瘤。随访结果:2例复发,1例颈部转移。结论鼻腔鼻窦畸胎癌肉瘤是一种由3胚层组成,具有畸胎瘤和癌肉瘤特征的罕见恶性肿瘤,具有特征性的组织病理学和免疫组织化学染色特点,应多取材多切片,以免误诊和漏诊。 Objective To investigate the clinicopathological features, diagnosis and differential diagnosis of nasal sinuses with teratocarcinoma. Methods The clinical, radiological and pathological data of 5 cases of pathologically confirmed nasal sinuses with teratocarcinoma were retrospectively analyzed. EnVision was performed on all the specimens and all the cases were followed up. Results 5 patients were male, aged 34 to 43 years, mean 39 years. The main clinical manifestations of unilateral nasal congestion, bloody headache and runny nose, nasal examination found that full of new creatures, the surface smooth, pedunculated, easy to touch the hemorrhage; radiology are local placeholder, uniform density, no obvious Cystic degeneration, calcifications and ossification. Pathological examination: the tumor mostly broken tissue, the texture is moderately tender, microscopic observation of the tumor often consists of three germ layers of the organization, the ectoderm squamous epithelium, mesodermal tumor-like hyperplasia of smooth muscle fibers, cartilage and bone tissue and within The germ layer is rich in mucilaginous ciliated columnar epithelium and arranged in the duct-like structure of the digestive tract, respiratory epithelium, in which immature neuroepithelial cells, cytoplasm transparent squamous cell mass, spindle cell sarcoma and adenocarcinoma most Diagnostic value. Immunohistochemical staining of different germ layers corresponding to the corresponding immune markers, cytokeratin, epithelial cell membrane antigen-labeled ectoderm stratified squamous epithelium, endodermal digestive and respiratory epithelium, vimentin, smooth muscle actin and other markers Spindle cell sarcoma area and smooth muscle tissue, S-100 protein, neuron-specific enolase, synaptophysin and other markers of immature neuroepithelial tissue, and nestin positive, CD99 labeled primary cells. The first pathological diagnosis of 5 patients were capillarinoma, olfactory neuroepithelioma, olfactory neuroblastoma, craniopharyngioma and malignant mixed tumor. Follow-up results: 2 cases of recurrence, 1 case of neck metastasis. Conclusion Nasal sinus teratoma Carcinosarcoma is a rare malignant tumor composed of three germ layers with features of teratoma and carcinosarcoma. It has characteristic features of histopathology and immunohistochemical staining. Multi-sections should be taken to avoid misdiagnosis And missed diagnosis.