Abstract: Objective To investigate the clinical manifestations, pathological features and pathogenesis of tumor-splenic sclerosing angiomatoid nodular transformation （SANT） syndrome. Methods The cases of spleen Sant cases with tumor in the Department of Pathology of Guangzhou Panyu Maternal and Child Health Hospital, Sun Yat Sen Memorial Hospital of Sun Yat sen University and the Department of Pathology of Guangzhou Huayin Medical Laboratory Center Co., Ltd. were retrospectively analyzed. The clinical data, pathological paraffin sections, immunohistochemistry and in situ hybridization sections were collected. A total of 22 case reports from 20 related articles in Wanfang database and PubMed medical database were searched and reviewed. Results Among the 8 cases of SANT, 4 had tumor-splenic SANT syndrome, which was found incidentally during the examination before tumor resection. The pathological features include the appearance of the SANT as a circular nodule with clear boundary and easy separation from the surrounding normal splenic tissue. There are a large number of mature sclerosing collagen fibers inside, forming segmented fibrous septa. Microscopically, the remnant red pulp tissue was surrounded by sclerosing fibrous collagen as an island, which was scattered with macrophages, lymphocytes, plasma cells and hemosiderin cells. There were different expression patterns of CD8, CD31 and CD34 in the three types of blood vessels. CD68 showed that macrophages in the residual red medullary were functionally active. Conclusion Both clinicians and pathologists need to consider the possibility of tumor-splenic SANT syndrome when diagnosing patients with SANT or malignant tumors with splenic mass.

Key words: tumor, sclerosing angiomatoid nodular transformation, syndrome, pathogenesis

摘要： 目的 探讨肿瘤-脾硬化性血管瘤样结节性转化（SANT）综合征的临床表现、病理学特征,并阐述其致病机制。方法 回顾性分析广州市番禺区妇幼保健院病理科、中山大学孙逸仙纪念医院病理科及广州华银医学检验中心有限公司病理科关于伴发肿瘤的脾SANT病例,收集其临床资料、病理石蜡切片、免疫组化及原位杂交切片。搜索并复习万方数据库和PubMed医学数据库中20篇相关文献共22例个案报道。结果 8例SANT病例中,4例为肿瘤-脾SANT综合征,患者均在进行肿瘤切除术前检查时偶然发现。病理特征包括：SANT外观呈类圆形结节,边界清晰,容易与周围正常的脾组织分离。其内部存在大量成熟的硬化性胶原纤维,形成分割性纤维间隔。镜下见残余红髓组织被硬化性纤维胶原围绕成岛状,其内散在巨噬细胞、淋巴细胞、浆细胞及含铁血黄素细胞。3种类型的血管在免疫组化CD8、CD31、CD34组合上存在不同的表达模式。CD68显示残余红髓内巨噬细胞功能活跃。结论 当临床医师和病理医师在诊断SANT患者或伴有脾脏占位的恶性肿瘤患者时,均需要考虑肿瘤-脾SANT综合征的可能。

关键词: 肿瘤, 脾硬化性血管瘤样结节性转化, 综合征, 致病机制