摘要
脾脏硬化性血管瘤样结节性转化（sclerosing angiomatoid nodular transformation，SANT）是一种少见、发病原因尚不清楚的脾脏良性病变。SANT具有独特的病理形态特点：境界清楚的实性肿块，多个血管瘤样结节为纤维硬化间质所包绕；血管瘤样区域对血管免疫标记显示出异质性；手术切除即可治愈。临床易将其误诊为其他类型肿瘤。通过复习SANT现有中、英文文献，从命名、临床及病理形态学特征、鉴别诊断、发病机制以及治疗与预后等方面总结现阶段对该病变的认识。
Sclerosing angiomatoid nodular transformation （SANT） is a rarely encountered benign spleen lesion which has recently been gaining recognition. However, the actual pathogenesis of this disease remains unclear. SANT＇s remarkably characteristic appearance consists grossly of multiple well circumscribed vascular/angiomatoid nodules of various sizes in a fibrosclerotic stroma. Immunohistochemical studies display a heterogeneous staining pattern of endothelial phenotypes in the angiomatoid nodules, with some cells resembling splenic sinusoids （CD34 -/CD31 ＋/CD8 ＋ ）, capillaries （CD34＋/CD31＋/CDS-） and small veins （CD34 -/CD31＋/CDS-）. Due to the rarity of this disease, it is often mistaken for splenic hamartoma, inflammatory pseudotumor, littoral cell angima or hemangioendothelioma. So far, SANT exhibits a benign clinical course because spleneetomy can be curative. In this review, we outline the clinical and pathologic features of SANT and discuss its probable disease mechanism to provide a holistic overview of the disease at this time.
出处
《中华肝胆外科杂志》
CAS
CSCD
北大核心
2012年第2期155-158,共4页
Chinese Journal of Hepatobiliary Surgery
关键词
脾脏疾病
免疫组化
发病机制
Splenic diease
Immunohistochemistry Pathogenesis
