Wang Hongqun, Hu Bingrong, Chen Wei, Ren Jiaming, Liu Peng, Shi Huaiyin
Department of Pathology, the Third People's Hospital of Bengbu City, Bengbu City, Anhui Province, 233000, China; Department of Pathology, the First Medical Center of the Chinese People's Liberation Army (PLA) General Hospital, Beijing, 100083, China.
Department of Pathology, the First Medical Center of the Chinese People's Liberation Army (PLA) General Hospital, Beijing, 100083, China; Department of Pathology, Lu'an People's Hospital, Lu'an City, Anhui Province, 237000, China.
Pathol Res Pract. 2021 Aug;224:153490. doi: 10.1016/j.prp.2021.153490. Epub 2021 May 24.
To explore the clinicopathological features of sclerosing angiomatoid nodular transformation (SANT) of the spleen.
The clinicopathological data of 26 SANT patients were analyzed.
There were 15 men and 11 women, aged 23-62 years (mean: 43.9 years; median: 43 years). Twenty patients were found during health check-ups. Magnetic resonance imaging had significantly higher specificity than other imaging modes in the diagnosis of SANT. Macroscopically, the lesions were gray-red and gray-white, along with well-demarcated nodules. Microscopy showed multiple angiomatoid nodules embedded in hyperplastic fibrous tissues and dense collagen fiber; the angiomatoid structures inside the nodules had varied morphology. Patchy and nodular fresh and old hemorrhages were observed in each lesion. Proliferative fibroblasts were seen in the stroma, along with infiltration of a few mixed inflammatory cells. Serum tumor markers were negative. Fourteen patients (53.8 %) had benign or malignant lesions in other parts of the body, including the liver, kidneys, and adrenal and pituitary glands which were similar to von Hippel-Lindau (VHL) syndrome. The reasons for occurrence of SANT may be as follows: hemangioma/lymphangioma or splenic congestion with extensive hemorrhage and secondary changes.
SANT is a rare benign vascular lesion with some clinical manifestations similar to VHL syndrome. Patients have good prognosis after tumor removal.
探讨脾脏硬化性血管瘤样结节性转化（SANT）的临床病理特征。
分析26例SANT患者的临床病理资料。
男性15例，女性11例，年龄23 - 62岁（平均43.9岁；中位数43岁）。20例患者在健康体检时被发现。磁共振成像在SANT诊断中的特异性显著高于其他成像方式。大体上，病变呈灰红色和灰白色，结节界限清晰。显微镜下可见多个血管瘤样结节包埋于增生的纤维组织和致密的胶原纤维中；结节内的血管瘤样结构形态各异。每个病变均可见斑片状及结节状新鲜和陈旧性出血。间质中可见增生的成纤维细胞，伴有少量混合性炎性细胞浸润。血清肿瘤标志物均为阴性。14例患者（53.8%）身体其他部位存在良性或恶性病变，包括肝脏、肾脏、肾上腺及垂体，类似于冯·希佩尔-林道（VHL）综合征。SANT的发生原因可能如下：血管瘤/淋巴管瘤或脾淤血伴广泛出血及继发性改变。
SANT是一种罕见的良性血管病变，部分临床表现类似于VHL综合征。肿瘤切除后患者预后良好。
