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眼眶原发性副神经节瘤的临床病理与发生学探讨 被引量:2

Clinicopathology and pathogenesis of primary paraganglioma of the orbit
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摘要 目的探讨眶部原发性副神经节瘤的临床病理特征及其与伴随的睫状神经和副神经节样组织的关系。方法复习病史及有关文献。采用HE、PAS、网状纤维(W ilder法)和免疫组化染色,观察肿瘤和邻近的副神经节样组织的形态和免疫表型特征。结果患儿女性,6岁。因左眼眼球突出,在某医院第一次行肿瘤切除后症状复发,于2000年8月住入安徽医科大学第一附属医院眼科,分别在8月和9月进行第二、三次手术。第三次剜出物为眶内容,3.5 cm×3.5 cm×2 cm,其中见一灰白色病灶,2.5 cm×1.0 cm。镜检:肿瘤有薄层纤维性包膜;包膜外结缔组织内有粗大的神经纤维分支及一团副神经节样组织;肿瘤实质由上皮样主细胞及短梭形支持细胞组成,间质为丰富的血窦样小血管,后者将实质细胞分隔成大小不一的实性细胞巢(细胞球)。PAS染色主细胞胞质内未见菱形或矩形或针状结晶。免疫组化显示:主细胞弥漫表达CgA、NSE、NF(中等阳性)和Syn(弱阳性),不表达CK、EMA、S-100蛋白、GFAP、HMB45、desm in及CD34,极少数vim entin阳性;支持细胞普遍表达vim entin,极少数表达S-100蛋白;瘤旁神经纤维强表达S-100蛋白、NF及NSE;副神经节样组织内大细胞强表达CgA、NSE和NF。病理学诊断副神经节瘤。术后随访2年,无复发,后失访。结论眶内原发性副神经节瘤极罕见。临床表现为眶内占位常引起的症状,无特异性,临床难以确诊。病理检查必须借助免疫组化和(或)电镜检查才能确诊。治疗以手术为主,可加用或单独选用放射治疗。40%病例可有复发,但无转移报道。本例瘤旁有睫状神经及一团副神经节(样)组织,提示此瘤与能分化为睫状副神经节之干细胞的异常分化有关。 Purpose To explore the clinic and pathologic characteristics of primary paraganglimoma of the orbit,and the relationship between the paraganglioma and associated with the ciliary nerve and paraganglia-like tissue. Methods The case history and the cases of literature were reviewed. The tumor was studied by HE, PAS, rcticulin( Wilder' s method) and immunohistochemistric stains. Results A 6-year-old girl was admitted the First Affiliated Hospital. Anhui Medical University ,on August ,2000 ,with the complaint of recurrent proptosis of the left eye. She was treated by first tumorectomy because same symptoms for six months at another Hospital three months ago. Secondary and last times tumorectomy were separately performed on August and September,2000. The last specimen consisted of a mass of tissue measuring 3.5 cm × 3.5 cm × 2 cm,which contained a grey lesion ,2. 5 cm × 1.0 cm. Microscopic finding:the tumor was surrounded by thin fibrous capsule, several nerve branches and a group of paraganglia-like tissue were presented in pericap- sule connective tissue. The parenchyma of tumor was composed of round to polygonal epithelial-like cell ( neoplastic chief cells ) and spindle sustentacular cells. These cells were arranged in small nests ( Zellballen ) surrounded by sinusoid vessels, showed" Organoid" architecture. These neoplastic chief cells were negative for PAS, and diffue moderately positive for CgA. NSE, NF and weakly for Syn, no chief cell immunoreactivity was detected for CK, EMA. S-100 protein GFAP,HMB45,desmin and CD34. The sustentacular cell were positive for vimentin ,and occasionally for S-100 protein. The large cells within the group of paraganglia-like tissue were strongly positive for CgA ,NSE and NF. Pathologic diagnosis was primary paraganglioma. Two years after last surgery, the patient was doing well without signs of tumor recurrence. Conclusions Primary paraganglioma of the orbit are extremely rare tumor. The clinical diagnosis is very difficult. The pathologic diagnosis must depends on hitsopathologic ,inanunohistochemistric and/or electron microscopical examination. Treatment includes surgical excision and radiation therapy. Almost 40% of orbital paraganglioma recur locally. There are no report of distand metastases. In this case, a group of paraganglia-like tissue near the capsule of tumor that suggests the tumor may possibly be relation with abnormal differentiation of commtted stem cell in situ.
出处 《临床与实验病理学杂志》 CAS CSCD 北大核心 2005年第5期577-582,共6页 Chinese Journal of Clinical and Experimental Pathology
关键词 眶肿瘤 副神经节瘤 睫状副神经节 病理学 临床 免疫组织化学 obital neoplasms paraganglioma ciliary paraganglia pathology, clinical immunohitochemistry
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