脾脏硬化性血管瘤样结节性转化临床病理分析并文献复习  

Sclerosing angiomatoid nodular transformation of spleen:a clinicopathological analysis and review of literature

作　　者：赵建民 梅雪[1] 刘伟[1] 张淑红[1] 郑媛媛[1] 柳玮华[1] 陈光勇[1] ZHAO Jian-min;MEI Xue;LIU Wei;ZHANG Shu-hong;ZHENG Yuan-yuan;LIU Wei-hua;CHEN Guang-yong(Department of Pathology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院病理科,北京100050

出　　处：《诊断病理学杂志》2022年第6期491-495,共5页Chinese Journal of Diagnostic Pathology

基　　金：国家自然科学基金面上项目(81972629);北京市医院管理中心消化内科学科协同发展中心专项经费资助(XXX0102)。

摘　　要：目的探讨脾脏硬化性血管瘤样结节性转化(sclerosing angiomatoid nodular transformation,SANT)的临床病理特征。方法分析8例SANT患者的临床影像表现、病理组织学特征、免疫组织化学特点及临床预后,并复习相关文献。结果男性3例,女性5例,年龄28~62岁,平均年龄46.7岁。6例无明显临床症状。大体检查:病变单发、边界尚清、无包膜,最大径为2.0~9.0cm,切面灰白色、红棕色、灰黄色,多结节状。镜下于纤维硬化间质内见多个血管瘤样结节,结节由窦样腔隙、圆形、不规则管腔的血管形成,管腔内衬肥胖内皮细胞,结节内及结节间散在不等量浆细胞、淋巴细胞、肌纤维母细胞及组织细胞浸润,免疫组织化学染色显示结节内含不同类型的小血管,血管衬覆内皮细胞的CD31、CD34、CD8表达有差异。随访17~98个月均无复发和转移。结论SANT现在认为是一种脾脏少见的良性血管增生性硬化性瘤样病变,具有特征性病理学形态及免疫组化特点,大部分患者无明显临床症状。确诊需手术后病理。脾脏切除可治愈,预后良好。Objective To explore the clinicopathological characteristics of sclerosing angiomatoid nodular transformation(SANT)of the spleen.Methods Clinical manifestation,radiological manifestation,pathomorphological characteristics,immunohistochemical features and prognosis of 8 cases of SANT were analyzed,and relevant literatures were reviewed.Results There were 3 males and 5 females in this study,whose age ranged from 28 years to 62 years(mean 46.7 years).Six of the cases showed no specific clinical manifestation.Grossly,the splenic lesion was solitary,well-demarcated,unencapsulated,measuring 2.0 cm to 9.0 cm in greatest diameter.The cut surface revealed a mass of gray white,red brown or gray yellow nodules.Microscopically,the lesions showed multiple angiomatoid nodules in a fibrosclerotic stroma.Each angiomatoid nodule was made up of slit-like,round,or irregular-shaped vascular spaces lined by plump endothelial cells.Scattered lymphocytes,plama cells,myofibroblasts and histiocytes were present in variable amounts within and outside the angiomatoid nodules.Immunohistochemical studies revealed different types of blood vessels within the angiomatoid nodules,the expression of CD31,CD34 and GD8 in vascular endothelial cells was different.No recurrence or metastasis was found during 17 months to 98 months of follow-up.Conclusion SANT is currently considered as a rare benign angioproliferative sclerosing tumor-like lesion,which has characteristic pathological morphology and immunohistochemical staining.Most patients have no obvious clinical manifestations.It can be only diagnosed on the basis of surgical histopathology.It could be cured by splenectomy with a favourable prognosis.

关 键 词：脾脏肿瘤 硬化性血管瘤样结节性转化 脾脏疾病 

分 类 号：R733.2[医药卫生—肿瘤]