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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BMC
2018-09-01
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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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institution | Directory Open Access Journal |
issn | 1897-4287 |
language | English |
last_indexed | 2024-12-13T07:57:30Z |
publishDate | 2018-09-01 |
publisher | BMC |
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series | Hereditary Cancer in Clinical Practice |
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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