摘要
目的探讨鼻腔鼻窦畸胎癌肉瘤(sinonasal teratocarcinosarcoma,SNTCS)的临床病理学特征、诊断、鉴别诊断、治疗及预后。方法回顾性分析10例SNTCS的临床病理特征及免疫表型等,并复习相关文献。结果10例患者中男性8例,女性2例,年龄28~69岁,平均47.3岁。10例患者肿块均位于鼻腔,多因鼻塞伴鼻出血、脓涕量增多伴嗅觉减退就诊。肿瘤大体多为碎组织,质嫩至质中;该肿瘤组织学形态多样,镜下可见胞质透明的鳞状细胞团、腺体及纤毛柱状上皮成分,部分腺上皮异型,梭形细胞间叶成分围绕未成熟的鳞状上皮及腺癌成分分布,部分病例可见分化不等的神经上皮细胞,肿瘤间质中可见软骨、骨样组织及平滑肌组织。不同胚层的组织免疫表型不同,鳞状上皮及腺上皮成分CK和EMA等上皮标志物阳性,间叶成分中vimentin阳性,分化成熟的神经组织对Syn、NSE、S-100蛋白表达敏感,原始神经外胚层细胞(特别是小圆形细胞)中CD99呈弥漫阳性,且Syn、NSE局灶阳性;desmin均呈阴性,仅1例SMA呈阳性;10例Ki-67增殖指数40%~60%。随访时间为1~93个月,截止2019年11月,6例患者存活。结论SNTCS属于罕见的具有高侵袭性生物学行为的恶性肿瘤,其由3个胚层组成,多处取材制片以及典型的组织病理学特征和免疫组化染色对诊断具有重要意义。
Purpose To study the clinicopathologic feature,diagnosis,differential diagnosis,treatment and prognosis of the sinonasal teratocarcinosarcoma(SNTCS).Methods The clinicopathologic features and immunotypes of 10 cases of SNTCS were retrospectively analyses,and the relevant literatures were reviewed.Results Among the 10 patients,there were 8 males and 2 females,aged from 28 to 69 years old,with an average of 47.3 years old.The mass were all located in the nasal cavity,mostly due to nasal obstruction with epistaxis,increased the nasal pus,and decreased sense of smell.The tumors were always fragmented,tender to medium,with a heterogeneous admixture of components histologically.Microscopically,the tumor was composed of squamous cell with clear cytoplasm,glandular epithelium and ciliated columnar epithelium.Some glandular epithelium cells were atypical,the spindle mesenchymal tissues were distributed around immature squamous epithelium and adenocarcinoma.The neuroepithelial cells with various differentiation were seen in some cases.The cartilage,osteoid tissue and smooth muscle tissue could be seen in the tumor stroma in some cases.The immunophenotypes of different germ layer were different,the epithelial markers such as CK and EMA were positively expressed in squamous epithelium and glandular epithelium cells,and the mesenchymal components expressed vimentin.The neuroepithelial components were sensitive to the expression of Syn,S-100 and NSE,while the primitive neuroectodermal cells(especially small round cells)diffusely expressed CD99,but focally expressed Syn and NSE.All the selected cases were negative for desmin,and only 1 case was positive for SMA,and the Ki-67 index of 10 cases was about 40%~60%.10 patients were followed up form 1 to 93 months.By the end of November 2019,6 patients were survived.Conclusion SNTCS is a rare malignant tumor with highly invasive biological behavior.It is consisted of 3 germ cell layers.Multiple sampling,histopathologic characteristics and its immunophenotypes are of great significant
作者
黄雯
吴海波
吴继锋
HUANG Wen;WU Hai-bo;WU Ji-feng(Department of Pathology,the First Affiliated Hospital of USTC,Division of Life and Sciences and Medicine,University of Science and Technology of China,Hefei230036,China;Department of Pathology,the Second Affiliated Hospital of Anhui Medical University,Hefei230601,China)
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2020年第5期534-537,共4页
Chinese Journal of Clinical and Experimental Pathology
基金
安徽省自然科学基金(1908085MH281)
中央高校基本科研业务费专项资金(WK9110000042)。
关键词
鼻肿瘤
畸胎癌肉瘤
诊断
免疫组织化学
nasal neoplasm
teratocarcinosarcoma
diagnosis
immunohistochemistry
