鼻腔鼻窦畸胎癌肉瘤3例临床病理分析并文献复习  

Clinicopathological Analysis of Three Cases of Sinonasal Teratocarcinosarcoma and Literature Review

作　　者：邬慧倩 刘燕萍 周爱君 李建明 WU Hui-qian;LIU Yan-ping;ZHOU Ai-jun;LI Jian-ming(Department of Pathology,Sun Yat-sen Memorial Hospital of Sun Yat-sen University,Guangzhou 510120,China)

机构地区：[1]中山大学孙逸仙纪念医院病理科,广州510120

出　　处：《循证医学》2024年第2期84-89,共6页The Journal of Evidence-Based Medicine

基　　金：国家自然科学基金重点项目(82130080)。

摘　　要：目的探讨鼻腔鼻窦畸胎癌肉瘤(sinonasal teratocarcinosarcoma,SNTCS)的临床病理特征,提高临床及病理医师对该疾病的认识。方法收集3例SNTCS的临床病理资料,采用EnVision二步法开展免疫组化检测,并结合相关文献,分析其临床病理特征。结果3例患者均为男性,年龄分别为35、55、69岁,肿瘤位于鼻腔、鼻窦,均表现为鼻塞、流脓涕。镜下可见良性及恶性上皮、间叶、神经上皮成分混杂分布,不同的成分表达相应的免疫组化标志物,其中2例显示所有成分均伴有SMARCA4缺失。1例仅行化疗,2例手术完整切除,其中1例术后放疗,1例术后联合放疗及化疗,随访时间4~20个月,其中2例分别于确诊后4个月和16个月失访,1例术后随访20个月仍存活。结论SNTCS组织学形态复杂多样,需与鼻腔鼻窦多种肿瘤鉴别,确诊依靠充分的取材。肿瘤恶性程度高,侵袭性强,尽早手术切除及术后放化疗可提高患者预后。Objective To investigate the clinicopathologic features of sinonasal teratocarcinosarcoma(SNTCS)in order to improve the understanding of clinician and pathologists.Methods The clinicopathological data of 3 cases of SNTCS were collected and immunohistochemistry was performed by EnVision two-step method,and related literature was reviewed.Results All the 3 cases were male,with the age of 35,55,and 69 years old(range 35~69),and located in the nasal cavity and sinuses,all of them presented with nasal obstruction and rhinorrhea.Microscopically,benign and malignant epithelial,mesenchymal.Different components expressed corresponding immunohistochemical markers,two cases showed that all components were SMARCA4 deficient.One case received chemotherapy only,two cases underwent complete surgical resection,including one case combined with radiotherapy and one case combined with radiotherapy and chemotherapy.The follow-up was 4~20 months,2 cases were lost to follow-up at 4 months and 16 months after diagnosis,respectively,and one case was still alive at 20 months after diagnosis.Conclusions The histological morphology of SNTCS is complex,which needs to be differentiated from various tumors of nasal cavity and sinuses,and the diagnosis depends on sufficient sampling.The tumor is highly malignant and aggressive.Early surgical resection and postoperative combination of radiotherapy and chemotherapy can improve the prognosis.

关 键 词：鼻肿瘤 畸胎癌肉瘤 鉴别诊断 病理分析 

分 类 号：R739.62[医药卫生—肿瘤]