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脾脏硬化性血管瘤样结节性转化

Sclerosing angiomatoid nodular transformation of the spleen.

作者信息

Awamleh Ahlam A, Perez-Ordoñez Bayardo

机构信息

Department of Pathology, University Health Network, Toronto General Hospital, 200 Elizabeth St, Toronto, Ontario, Canada.

出版信息

Arch Pathol Lab Med. 2007 Jun;131(6):974-8. doi: 10.5858/2007-131-974-SANTOT.

DOI:10.5858/2007-131-974-SANTOT
PMID:17550330
Abstract

Sclerosing angiomatoid nodular transformation (SANT) is a recently recognized nonneoplastic vascular lesion of the spleen with fewer than 30 cases described. Microscopically, SANT consists of multiple well-circumscribed vascular/angiomatoid nodules showing plump endothelial cell and extravasated erythrocytes. The nodules are surrounded by a variable lymphoplasmacytic infiltrate, spindle cells, and collagenous stroma. The vascular nodules display a complex mixture of endothelial phenotypes resembling splenic sinusoids (CD34-/CD31+/CD8+), capillaries (CD34+/ CD31+/CD8-), and small veins (CD34-/CD31+/CD8-). Focal expression of CD68 can also be seen. The differential diagnosis of SANT includes splenic hamartoma, inflammatory myofibroblastic tumor, littoral cell angioma, and hemangioendothelioma. It has been postulated that SANT represents a peculiar hamartomatous transformation of splenic red pulp in response to an exaggerated nonneoplastic stromal proliferation. SANT has a benign clinical course with splenectomy being curative.

摘要

硬化性血管瘤样结节性转化(SANT)是一种最近才被认识的脾脏非肿瘤性血管病变,报道的病例少于30例。在显微镜下,SANT由多个边界清晰的血管/血管瘤样结节组成,这些结节可见饱满的内皮细胞和外渗的红细胞。结节被不同程度的淋巴细胞和浆细胞浸润、梭形细胞及胶原性间质所包绕。血管结节呈现出复杂的内皮细胞表型混合,类似于脾血窦(CD34-/CD31+/CD8+)、毛细血管(CD34+/CD31+/CD8-)和小静脉(CD34-/CD31+/CD8-)。CD68也可见局灶性表达。SANT的鉴别诊断包括脾错构瘤、炎性肌成纤维细胞瘤、边缘细胞血管瘤和血管内皮瘤。据推测,SANT代表脾红髓对非肿瘤性基质过度增殖的一种特殊错构瘤样转化。SANT临床过程良性,脾切除术可治愈。

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