鞍区原发性脉络丛乳头状瘤临床病理观察

Primary choroid plexus papilloma of sellar region:a clinicopathologic analysis

目的探讨鞍区原发性脉络丛乳头状瘤的临床病理特征。方法对1例原发于鞍区的脉络丛乳头状瘤的影像学、组织病理学和免疫组化特征进行研究,结合文献讨论其临床病理特征及鉴别诊断。结果磁共振成像显示蝶鞍增大,其内可见团块状肿块,侵及鞍上池;脑室系统无异常。内镜经鼻蝶入路行肿物切除术。镜下观察可见肿瘤组织类似于正常脉络丛,呈乳头状结构,由单层或假复层柱状肿瘤上皮细胞围绕纤维血管轴形成。肿瘤细胞异型性小,细胞核圆或卵圆形,位于上皮基底部。免疫组化显示肿瘤细胞CK-pan、vimtentin、EMA和TTF-1(+),而S-100蛋白、神经胶质酸性蛋白、Syn和嗜铬粒蛋白(-)。结论原发于鞍区的脉络丛乳头状瘤非常罕见,影像学易与垂体腺瘤等相混淆。明确诊断依赖于病理组织学观察及免疫组化标记。外科手术完整切除可治愈。

Objective To study the clinicopathological features of primary choroid plexus papillomas in the sellar region.Methods Radiographical pattern,histological observation and immunohistochemical studies were conducted in a case of primary choroid plexus papillomas of the sellar region,and its clinical findings,histopathologic features and differential diagnosis were discussed in combination with review of the literature.Results Magnetic resonance imaging demonstrated a mass in the sellar region,which occupied the enlarged sella turcica and extended to the suprasellar cistern.No abnormality was found in the ventricular system.The mass was excised via an endonasal transsphenoidal approach.Histologic examination indicated a tumor had a papillary structure covered with one or more layers of columnar epitheial cells around a fibrovascular core that resembled normal choroid plexus.The tumor cells were well differentiated with round or ovoid nuclei situated in the basement of them.Immunohistiochemistry showed that the tumor was positive for cytokeratin-pan,vimentin,epithelial membrane antigen,thyroid transcription factor-1,and negative for glial fibrillary acidic protein,S-100 protein,synaptophysin,and chromogranin.Conclusion Choroid plexus papillomas arising in and occupying the sella are rare and it is extremely difficult to distinguish radiologically from pituitary adenoma.The diagnosis mainly relies on histopathology and immunohistochemistry.It can be cured by completely surgical excision.