盆腹腔原发性室管膜瘤临床病理观察及文献复习  

Primary ependymoma in pelvic and abdominal cavity:a clinicopathological analysis and review of literature

作　　者：张煜涵 王征 郭丽娜[1] 白春梅[2] 陆俊良[1] 霍真[1] ZHANG Yu-han;WANG Zheng;GUO Li-na;BAI Chun-mei;LU Jun-liang;HUO Zhen(Department of Pathology;Department of Medical Oncology,Chinese Academy of Medical Sciences,Pehing Union Medicial College,Peking Union Medicial College Hospital,Beijing 100730,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院病理科,北京100730 [2]中国医学科学院北京协和医学院北京协和医院肿瘤内科,北京100730

出　　处：《诊断病理学杂志》2024年第5期410-413,共4页Chinese Journal of Diagnostic Pathology

基　　金：中央高水平医院临床科研业务费资助(项目编号:2022-PUMCH-B-063)。

摘　　要：目的 总结盆腹腔原发性室管膜瘤的临床病理特征、诊断要点及治疗和预后情况。方法 总结经北京协和医院会诊确诊的1例及文献报道的共30例盆腹腔原发室管膜瘤病例。结果 本例患者女性,34岁,B超显示左侧髂窝边界清楚的弱回声团块,于外院行肿瘤细胞减灭术,术中见左髂窝实性肿物,包膜完整,质地中等。镜下见肿瘤组织排列成筛管状、实性、乳头状及腺样,可见菊形团,核分裂象罕见。免疫组化肿瘤细胞EMA、D2-40呈核旁点状阳性,GFAP、ER、PR、PAX-8、S-100和WT-1不同程度阳性。结论 盆腹腔原发室管膜瘤十分罕见,多见于年轻女性,误诊率高,ER、PR、WT-1和PAX-8免疫组化有较高的表达率。治疗以手术及术后化疗为主,肿瘤易复发,但死亡率低。Objective To provide a comprehensive overview of clinicopathological features, differential diagnosis, treatment, and prognosis of primary ependymoma in the pelvic and abdominal cavity. Methods This study summarizes one consultation case diagnosed in Peking Union Hospital and reviews 30 cases of primary ependymoma in the pelvis and abdominal cavity from the literature. Results We presented a 34-year-old woman with a well-defined weak echo mass in the left iliac fossa detected through B-ultrasound examination. The patient underwent cytoreductive surgery in another medical facility. During surgical exploration, a solid mass was found in the left iliac fossa that had a complete envelope and medium texture. Upon microscopic examination, the tumor exhibited a unique arrangement characterized by a sieve-like, solid, papillary, and adenoid pattern. Ependymal rosettes and rare mitotic figures were also observed. Immunohistochemical analysis revealed a dot-like perinuclear expression for EMA and D2-40 in the tumor cells, along with varying degrees of positivity for GFAP, ER, PR, PAX-8, S-100, and WT-1. Conclusion Primary pelvic and abdominal ependymomas are rare and typically observed in young women, which may lead to misdiagnosis. Immunohistochemistry typically exhibits positive results for ER, PR, WT-1, and PAX-8. Common treatments include surgery and adjuvant chemotherapy. While this tumor tends to recur, the associated mortality rate remains low.

关 键 词：盆腹腔 室管膜瘤 病理特征 免疫组化 临床表现 

分 类 号：R739.9[医药卫生—肿瘤]