肾脏及肾上腺交织状血管瘤临床病理分析
Clinicopathologic analysis of anastomosing hemangioma of the kidney and adrenal gland
摘要目的:探讨交织状血管瘤的临床特点、组织病理学特征、诊断和鉴别诊断要点以及生物学行为。方法收集5例肾脏和肾上腺的交织状血管瘤,观察其临床及影像学特点,组织形态学和免疫表型以及预后,并结合相关文献进行总结和复习。结果5例肿瘤3例男性,2例女性,发病年龄47~77岁;3例肿瘤位于肾上腺,2例位于肾脏。临床上4例肿瘤为偶然发现,1例肾上腺肿瘤表现为双下肢水肿;影像学上均表现为界限清楚的、类圆形的实性低密度影肿块。大体上肿瘤最大直径从1.6~2.5 cm(平均2.1 cm)。组织学上5例肿瘤均由交织索状的脾窦样血管腔构成,被覆为单层的扁平、立方至鞋钉样内皮细胞,分布于少细胞的玻璃样或纤维性间质之中;其他常见的组织学特征包括:模糊的分叶状结构(3/5);血管内出血伴血栓沉积(5/5),肿瘤于血管腔内生长(5/5),瘤细胞胞质内嗜酸性小球聚集(1/5),髓外造血(3/5)。瘤细胞大多数形态温和,核分裂象罕见;1例肿瘤局灶区富于细胞伴轻度的多形性(核分裂象约2个/50 HPF)。免疫组织化学染色显示肿瘤表达血管内皮细胞标志物。5例肿瘤均获得随访资料,随访6~52个月(平均约30个月)均未见复发和转移的证据。结论交织状血管瘤是一种罕见的良性血管瘤亚型,好发于泌尿生殖器官和肾上腺,在形态学上需要与一系列富于血管的良恶性肿瘤区分,仔细的形态学观察并适当的应用免疫组织化学标志物通常可将其准确的区分开来。
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abstractsObjective To investigate the clinical and histopathologic characteristics, diagnosis, differential diagnosis and prognostic features of anastomosing hemangioma.Methods Five cases of anastomosing hemangioma of the kidney and adrenal gland were collected, the clinical and radiologic features, histomorphology, immunophenotype and prognosis were analyzed with review of literature.Results Three patients were male and two were female with ages ranging from 47 to 77 years; three were located in adrenal gland and 2 originated from the kidney.Clinically, 4 tumors were incidentally identified, 1 presented as edema of lower extremity.By radiography, all presented as a well-demarcated, oval, solid and low-density mass.Grossly, the tumors ranged in maximum diameter from 1.6 to 2.5 cm ( mean 2.1 cm) .Microscopically, the tumors consisted of anastomosing sinusoidal capillary-sized vessels lined by a single layer of flattened, cubical to hobnail endothelial cells, setting in an pauci-cellular stroma of edematous and hyaline changes.Other commonly seen features included vaguely lobular growth pattern ( 3/5 ) , hemorrhage and thrombosis ( 5/5 ) , intravascular growth pattern ( 5/5 ) , eosinophilic intracytoplasmic hyaline globules ( 1/5 ) and extramedullary hematopoiesis ( 3/5 ).The tumor cells were typically bland-appearing and mitoses were scarce, with 1 case demonstrating cellular foci of tumor with slight pleomorphism and increased mitoses ( 2/50 HPF ).Immunohistochemical studies showed the tumor cells expressed endothelial cells markers.Follow-up information was available for all 5 patients and showed no evidence of tumor recurrence or metastasis within 6 to 52 months ( mean 30 months).Conclusions Anastomosing hemangioma is a rare, benign subtype of capillary hemangioma that predominantly affects the urologic organs and adrenal glands;it is needed to distinguish it histologically from a series of benign or malignant tumors that feature a richly vascular stroma.Careful attentions to its characteristic morphology with the judicious use of immunohistochemistry can help distinguish this tumor from its many mimickers.
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