鼻腔鼻窦畸胎癌肉瘤(sinonasal teratocarcinosarcoma,SNTCS)非常罕见,目前在国内外文献中均缺乏大宗的病例报道。SNTCS的临床特点为患者以中老年男性较多见,肿瘤最常发生于鼻腔和筛窦,偶尔可位于上颌窦、蝶窦及鼻咽部,有时肿瘤可浸润至颅底部。显微镜下见肿瘤内的成分非常复杂,在肿瘤内可见不同分化程度来源于3个胚层的组织,并混有癌及肉瘤成分。SNTCS易与多种其他肿瘤相混淆,其鉴别诊断常很困难。SNTCS的生物学行为多呈高度恶性,肿瘤生长十分迅速,并有很强的侵袭性,肿瘤易发生局部淋巴结及远处转移,患者的预后较差。作者着重对SNTCS的组织学起源、临床及病理学特点、病理诊断、鉴别诊断及患者的预后等作一综述。 Sinonasal teratocarcinosarcoma (SNTCS) is very rare. There are currently no large number of cases reported in domestic and foreign literature. The clinical features of SNTCS are more common in middle-aged and elderly men. Tumors usually occur in the nasal cavity and ethmoid sinus. Occasionally, they can be located in the maxillary sinus, sphenoid sinus and nasopharynx. Sometimes the tumor infiltrates to the base of the skull. Microscopic tumor composition is very complex, visible in the tumor differentiation of the degree of differentiation from the three germ layers of the organization, and mixed with cancer and sarcoma components. SNTCS easily confused with a variety of other tumors, the differential diagnosis is often difficult. Most of the biological behaviors of SNTCS are highly malignant, the tumor growth is very rapid, and has a strong invasiveness, the tumor prone to local lymph nodes and distant metastasis, the prognosis of patients is poor. The author focuses on the histological origin of SNTCS, clinical and pathological features, pathological diagnosis, differential diagnosis and prognosis of patients were reviewed.