Abstract
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.
Highlights
Hemangioblastomas are associated with elevated hemoglobin (Hb) levels, which is associated with a higher risk for cerebral stroke, cardiac infarction and pulmonary embolism
It results in a higher risk to develop thrombosis, which can lead to severe complications such as cerebral stroke, cardiac infarction and pulmonary embolism
To elucidate the correlations of possible causative factors such as EPO levels, tumor size and cystic formations, co-morbidities and von Hippel–Lindau (VHL) mutation specificity in patients with hemangioblastomas, we systematically investigated a consecutive series of patients operated on hemangioblastomas in our department
Summary
Hemangioblastomas 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. Hemangioblastomas are highly vascular neoplasms, which can occur as sporadic tumors or as part of the von Hippel–Lindau (VHL) disease [1]. They comprise approximately 3% of all tumors of the central nervous. We and others have more recently assessed the incidence much lower with numbers around 5–8% [12]
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