摘要
目的探讨婴儿鼻软骨间叶性错构瘤(nasal chondromesenchymal hamartoma,NCMH)的临床病理特征及鉴别诊断要点。方法对2例NCMH的临床资料、组织学形态和免疫表型进行分析,并复习相关文献。结果 2例男婴均<1岁,表现为鼻塞或鼻腔占位及呼吸不畅。CT和MRI示鼻腔鼻窦占位并累及眼眶等。两者均于内镜下行鼻腔鼻窦新生物摘除术。镜下见肿瘤位于黏膜下,由不规则岛状软骨结节和疏密不均的梭形细胞组成,间质伴黏液样变性,并见相互混杂的胶原纤维。例2中有多灶钙化。局部形态类似结节性筋膜炎或纤维组织细胞瘤。免疫组化标记软骨结节表达S-100蛋白,梭形细胞主要表达vim-entin。2例患者术后均恢复良好。结论 NCMH是一种好发于婴幼儿鼻腔鼻窦的良性错构瘤性病变,研究NCMH的临床及病理学特征有助于对该病正确认识,避免误诊及不适当的治疗。
Purpose To explore the clinicopathological features of nasal chondromesenchymal hamartoma （NCMH） and its differential diagnosis. Methods The clinical data, histological features and immunophenotype of 2 NCMH cases were analyzed , and the litera- ture was reviewed. Results Both of the patients were boys within 1 -year-old, and admitted to the hospital with nasal obstruction or oc- cupation in nasal cavity and obstructed breathing. CT and MRI detection confirmed large occupation in nasal cavity, which also in- volved the sinus and fossa orbitalis. The neoplasm was surgically excised through endoscopy for both patients. Microscopically, the tumor was under the mucosa, and composed of irregular lobules or islands of benign hyaline cartilage surrounded by spindle cell stro- ma. Spindle mesenchymal elements varied from loose to compact, with myxoid change, and mixed with collagen fibers. In addition, multiple foci of distinguished calcification were seen in case 2. Focal areas resembling nodular fasciitis or fibrohistocytoma were pres- ent. The immunohistochemical study showed the cartilage nodules, as well as some spindle cells around the nodules, were positive for S-100 protein. The spindle cells predominantly expressed vimentin. Both patients were free of any symptoms and recovered well at the last contact. Conclusion NCMH is a benign harmatomatous lesion mainly occurred in the nasal cavity and nasal sinuses of infancy, and being well familiar with the clinical features and pathological characteristics will be of great help to a better understanding of this disease, and avoid misdiagnosis and potentially harmful therapies.
出处
《临床与实验病理学杂志》
CAS
CSCD
北大核心
2013年第6期649-655,共7页
Chinese Journal of Clinical and Experimental Pathology
关键词
鼻腔肿瘤
错构瘤
病理学
临床
婴儿
nasal cavity neoplasms
hamartoma
pathology
clinical
infancy
