Department of Pathology, Ohio State University Medical Center, Columbus, Ohio, USA.
Neuropathology. 2010 Dec;30(6):580-5. doi: 10.1111/j.1440-1789.2010.01109.x.
Hemangioblastomas (HBs) account for nearly a tenth of all posterior fossa neoplasms and can be the presenting finding in patients with von Hippel-Lindau (VHL) syndrome. HB must be differentiated from renal cell carcinoma (RCC), also seen in VHL, as the distinction between these lesions dictates the management of these patients. Currently inhibin A and RCC marker have been used in the diagnosis of HB and metastatic RCC, both with inconsistent results. Additional immunohistochemical markers including CD10, PAX-2, D2-40, and FLi-1 have been shown to have potential for the distinction of these two entities. Fifteen cerebellar HBs and 17 metastatic clear cell RCCs to the brain were selected for the study. All cases were immunostained with RCC marker, inhibin, CD10, PAX-2, D2-40, and Fli-1. The staining patterns were scored based on intensity and extent of tumor staining. In the differentiation of HB and metastatic RCC, D2-40 and RCC marker proved to be poor markers with less than 50% of HBs and RCCs, respectively, showing positive staining. PAX-2 and CD10 were superior to RCC marker in the diagnosis of metastatic RCC, with PAX-2 having better specificity. Fli-1 failed to stain tumor cells in both HBs and RCC. Inhibin A, in combination with PAX-2, showed to be the most useful markers to differentiate HB from metastatic RCC.
血管母细胞瘤（HBs）占后颅窝肿瘤的近十分之一，并且可能是 von Hippel-Lindau（VHL）综合征患者的首发表现。HB 必须与 VHL 中也可见的肾细胞癌（RCC）相区别，因为这些病变之间的区别决定了这些患者的治疗方案。目前，抑制素 A 和 RCC 标志物已用于 HB 和转移性 RCC 的诊断，但结果不一致。其他免疫组织化学标志物，包括 CD10、PAX-2、D2-40 和 FLi-1，已显示出用于区分这两种实体的潜力。选择了 15 例小脑 HBs 和 17 例转移性透明细胞 RCC 到脑部进行研究。所有病例均用 RCC 标志物、抑制素、CD10、PAX-2、D2-40 和 FLi-1 进行免疫染色。根据肿瘤染色的强度和范围对染色模式进行评分。在 HB 和转移性 RCC 的鉴别中，D2-40 和 RCC 标志物的阳性染色率均低于 50%，表明其作为标志物的效果不佳。PAX-2 和 CD10 在诊断转移性 RCC 方面优于 RCC 标志物，其中 PAX-2 的特异性更好。FLi-1 未能在 HBs 和 RCC 中染色肿瘤细胞。抑制素 A 与 PAX-2 联合使用，是鉴别 HB 与转移性 RCC 的最有用标志物。
