Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin

Abstract Background 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 postula...

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Main Authors: Marie T. Krüger, Jan-Helge Klingler, Cordula Jilg, Christine Steiert, Stefan Zschiedrich, Vera Van Velthoven, Sven Gläsker
Format: Article
Language:English
Published: BMC 2018-09-01
Series:Hereditary Cancer in Clinical Practice
Online Access:http://link.springer.com/article/10.1186/s13053-018-0097-x
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author Marie T. Krüger
Jan-Helge Klingler
Cordula Jilg
Christine Steiert
Stefan Zschiedrich
Vera Van Velthoven
Sven Gläsker
author_facet Marie T. Krüger
Jan-Helge Klingler
Cordula Jilg
Christine Steiert
Stefan Zschiedrich
Vera Van Velthoven
Sven Gläsker
author_sort Marie T. Krüger
collection DOAJ
description Abstract Background 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. Methods To 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. Results As 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. Conclusions We 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.
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spelling doaj.art-18b6669fcf4d44f3861813c453a5efe62022-12-21T23:54:31ZengBMCHereditary Cancer in Clinical Practice1897-42872018-09-011611710.1186/s13053-018-0097-xPolyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietinMarie T. Krüger0Jan-Helge Klingler1Cordula Jilg2Christine Steiert3Stefan Zschiedrich4Vera Van Velthoven5Sven Gläsker6Department of Neurosurgery, Freiburg University Medical CenterDepartment of Neurosurgery, Freiburg University Medical CenterDepartment of Urology, Freiburg University Medical CenterDepartment of Neurosurgery, Freiburg University Medical CenterDepartment of Internal Medicine, Section for Preventive Medicine, Freiburg University Medical CenterDepartment of Neurosurgery, Universitair Ziekenhuis BrusselDepartment of Neurosurgery, Freiburg University Medical CenterAbstract Background 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. Methods To 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. Results As 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. Conclusions We 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.http://link.springer.com/article/10.1186/s13053-018-0097-x
spellingShingle Marie T. Krüger
Jan-Helge Klingler
Cordula Jilg
Christine Steiert
Stefan Zschiedrich
Vera Van Velthoven
Sven Gläsker
Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin
Hereditary Cancer in Clinical Practice
title Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin
title_full Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin
title_fullStr Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin
title_full_unstemmed Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin
title_short Polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin
title_sort polyglobulia in patients with hemangioblastomas is related to tumor size but not to serum erythropoietin
url http://link.springer.com/article/10.1186/s13053-018-0097-x
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