BackgroundHemangioblastomas are associated with elevated hemoglobin (Hb) levels (polyglobulia), which is associated with a higher risk for cerebral stroke, cardiac infarction and pulmonary embolism. The pathomechanism of polyglobulia remains unclear and different theories have been postulated. Among those are elevated serum erythropoietin (EPO) levels caused by secretion of the tumor or associated tumor cyst.MethodsTo elucidate the pathomechanism, we systematically investigated the relation between polyglobulia, serum EPO level, size of the solid tumor and associated cyst in hemangioblastomas. We prospectively evaluated hemoglobin and EPO levels in a series of 33 consecutive patients operated on hemangioblastomas in our center. We measured the size of the solid tumor and associated cyst in magnetic resonance imaging. Statistical evaluations were performed using the Fisher’s exact test and student’s t-test.ResultsAs a result five patients had elevated hemoglobin levels. Only one of these had an elevated serum EPO level. Of 26 patients with normal hemoglobin levels, 4 patients had elevated EPO levels.Patients with low or normal hemoglobin levels (84%) had an average tumor size of 0.8 cm3, which differed significantly from patients with elevated hemoglobin levels (16%), who had an average solid tumor size of 8.0 cm3 (p < 0.05). We did not observe a significant correlation between EPO levels or polyglobulia and associated cysts.ConclusionsWe therefore conclude that in contrast to previous case reports and interpretations, our data show no correlation between polyglobulia and EPO levels or associated cysts in patients with hemangioblastomas. In fact, it is the size of the solid tumor that correlates with polyglobulia.The study was retrospectively registered in the German Clinical Trial Registry on 10 July 2014; Trial registration: DRKS00006310.

中文翻译：

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血管母细胞瘤患者的多球体与肿瘤大小有关，但与血清促红细胞生成素无关

背景血管母细胞瘤与血红蛋白 (Hb) 水平升高（多球）相关，这与脑卒中、心肌梗塞和肺栓塞的风险增加有关。多球菌的发病机制仍不清楚，并提出了不同的理论。其中包括由肿瘤分泌或相关肿瘤囊肿引起的血清促红细胞生成素（EPO）水平升高。方法为阐明发病机制，我们系统地研究了血管母细胞瘤中多球、血清EPO水平、实体瘤大小和相关囊肿之间的关系。我们前瞻性地评估了在我们中心接受血管母细胞瘤手术的 33 名连续患者的血红蛋白和 EPO 水平。我们在磁共振成像中测量了实体瘤和相关囊肿的大小。使用Fisher 精确检验和学生t 检验进行统计评估。结果5 名患者的血红蛋白水平升高。其中只有一个具有升高的血清 EPO 水平。在 26 名血红蛋白水平正常的患者中，4 名患者的 EPO 水平升高。血红蛋白水平低或正常的患者 (84%) 的平均肿瘤大小为 0.8 cm3，与血红蛋白水平升高的患者 (16%) 有显着差异。平均实体瘤大小为 8.0 cm3 (p < 0.05)。我们没有观察到 EPO 水平或多球蛋白与相关囊肿之间的显着相关性。结论我们因此得出结论，与以前的病例报告和解释相反，我们的数据显示血管母细胞瘤患者的多球蛋白和 EPO 水平或相关囊肿之间没有相关性。实际上，与多球体相关的是实体瘤的大小。该研究于 2014 年 7 月 10 日在德国临床试验注册中心进行了回顾性注册；试用注册：DRKS00006310。