North P E, Mizeracki A, Mihm M C, Mrak R E
Department of Pediatric Pathology, Arkansas Children's Hospital, Little Rock, Arkansas 72202-3591, USA.
Clin Neuropathol. 2000 May-Jun;19(3):131-7.
Hemangioblastoma and metastatic renal cell carcinoma (RCC) may show striking histologic similarities, and the distinction between these two tumors can be difficult. Both occur in middle age, and both occur with increased incidence in von Hippel-Lindau disease (vHL). GLUT1 is an erythrocyte-type glucose transporter protein that is highly expressed by endothelia in brain--but not most peripheral--microvasculature, and by tumor cells in many epithelial malignancies. GLUT1 is expressed by endothelial cells in juvenile hemangiomas, and endothelial GLUT1 expression has been reported for 2 hemangioblastomas arising in a single patient with vHL.
We performed immunoreactions for GLUT1 on archival hemangioblastomas from 12 patients (one with vHL), and on RCCs metastatic to brain of 9 patients.
Hemangioblastomas showed intense endothelial GLUT1 reactivity in 11/12 tumors resections; the only GLUT1-negative tumor was one for which only previously frozen material was available for immunoreaction, and this tissue showed poor GLUT1 immunoreactivity of internal erythrocyte controls. Hemangioblastoma stromal cell reactivity was found in only 1 case, and was weak and focal. RCCs, in contrast, showed no intralesional endothelial GLUT1 reactivity, but did show intense tumor cell membrane reactivity in 9/9 cases.
that GLUT1 immunoreactivity patterns reliably distinguish hemangioblastoma from RCC.
血管母细胞瘤与转移性肾细胞癌(RCC)在组织学上可能表现出显著相似性,区分这两种肿瘤可能存在困难。两者均发生于中年,且在冯·希佩尔-林道病(vHL)中的发病率均有所增加。葡萄糖转运蛋白1(GLUT1)是一种红细胞型葡萄糖转运蛋白,在脑微血管内皮细胞(而非大多数外周微血管内皮细胞)以及许多上皮性恶性肿瘤的肿瘤细胞中高表达。GLUT1在幼年血管瘤的内皮细胞中表达,并且有报道称在一名患有vHL的患者身上出现的2例血管母细胞瘤中存在内皮GLUT1表达。
我们对12例患者(其中1例患有vHL)存档的血管母细胞瘤以及9例脑转移RCC进行了GLUT1免疫反应检测。
在12例肿瘤切除标本中的11例,血管母细胞瘤显示出强烈的内皮GLUT1反应性;唯一的GLUT1阴性肿瘤是仅有的可用于免疫反应的先前冷冻材料的肿瘤,并且该组织显示内部红细胞对照的GLUT1免疫反应性较差。仅在1例中发现血管母细胞瘤基质细胞反应性,且反应较弱且呈局灶性。相比之下,RCC在瘤内未显示内皮GLUT1反应性,但在9/9例中确实显示出强烈的肿瘤细胞膜反应性。
GLUT1免疫反应模式可可靠地区分血管母细胞瘤与RCC。