目的 探讨脾硬化性血管瘤样结节性转化(SANT)的临床病理特征。方法 收集2016-09至2022-09解放军总医院第四、六医学中心SANT患者的临床资料,主要分析其临床病理特征,同时分析影像学特征、免疫组化等资料。结果 共7例,其中男5例,女2例,年龄32~49岁,平均42.3岁,大部分偶然发现;6例单发,最大径3.0~14.5 cm(平均7.4 cm);1例多发,最大者5.7 cm×5 cm×3.5 cm,最小者0.7 cm×0.6 cm×0.6 cm;镜下见多个由纤维包绕的血管瘤样结节,结节内血管成分复杂,呈脾窦样、毛细血管样或静脉样,血管内皮细胞的CD34、CD31、CD8表达有所差异。周围梭形细胞SMA表达灶状阳性。原位杂交EBER检测结果均为阴性。结论 SANT是一种少见的具有独特病理学特征的良性血管性病变,多无临床症状,易误诊,手术切除即可治愈,术后病理学形态和免疫组织化学染色是明确诊断的重要方法。
Abstract
Objective To investigate the clinicopathological features of sclerosing angiomatoid nodular transformation(SANT). Methods Clinical data of SANT patients in the Fourth and Sixth Medical Center of PLA General Hospital from 2016 to 2022 were collected, and the clinicopathological features, imaging features and immunohistochemical data were analyzed. Results A total of 7 cases were found, including 5 males and 2 females, aged 32-49 years with an average age of 42.3 years. Six cases were single, with a maximum diameter of 3.0-14.5 cm (average 7.4 cm), and 1 case was multiple, with a maximum diameter of 5.7 cm×5 cm×3.5 cm, with a minimum diameter of 0.7 cm×0.6 cm×0.6 cm. Microscopically, there were multiple hemangiomatous nodules surrounded by fibers. The vascular components in the nodules were complex, which were like splenic sinus, capillary or venous. The expressions of CD34, CD31, and CD8 in vascular endothelial cells were different. The SMA expression in peripheral spindle cells was focally positive. EBER detection results of in situ hybridization were negative. Conclusions SANT is a rare benign vascular lesion with unique pathological features, which has no clinical symptoms and is easy to be misdiagnosed. It can be cured by surgical resection. Postoperative pathological morphology and immunohistochemical staining are important methods for definite diagnosis.
关键词
脾脏 /
脾硬化性血管瘤样结节性转化 /
临床病理 /
鉴别诊断
Key words
spleen /
sclerosing angiomatoid nodular transformation /
clinical pathology /
differential diagnosis
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