Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review

Jose Carlos Rodrigues Junior; Vithor Ely Bortholin da Silva; Roberto Bezerra Vital; Renan Luiz Lapate; Marcelo Ferraz Campos

doi:doi:10.11648/j.ijn.20210501.11

Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review

Jose Carlos Rodrigues Junior, Vithor Ely Bortholin da Silva, Roberto Bezerra Vital, Renan Luiz Lapate, Marcelo Ferraz Campos

Published in International Journal of Neurosurgery (Volume 5, Issue 1)

Received: 27 April 2020 Accepted: 19 August 2020 Published: 4 January 2021

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Abstract

Background and Importance: Hemangioblastomas (HBL) are tumors constantly present in the neurosurgeon's daily life, affecting mainly the posterior fossa region. In patients with von Hippel-Lindau, their presence is even more striking. However, the authors, through a report of a case with the literature review, show that hemangioblastomas should also be part of the range of differential diagnoses of supratentorial lesions even in patients not carrying the genetic mutation. Clinical Presentation: A 45-year-old male patient has a 2 weeks history of headache associated with nauseas and vertigo. In the last two weeks the pain was being progressively more frequently with increase in intensity and duration. At examination he was oriented, with face of pain, muscle examination was normal as all the neurological examination. The patient was submitted to a Magnetic Resonance (MR) of the brain that showed an expansive lesion in the brain located in the left frontal lobe, adjacent to the medial line with moderated adjacent edema and with gadolinium injection the tumor was heterogeneous almost solid with cystic parts and including adjacent impregnation of the dura of the midline. Surgical treatment was done. The lesion was completed resected including the dura of the convexity that was invaded by the tumor. Postoperative period was uneventful, with resolution of the headache and no deficits. Patient was discharged 3 days after surgery. Pathology reveals the lesion was a hemangioblastoma. Patient made genetic exams and confirms that didn’t have Von Hippel Lindau disease. MR of the brain, almost 3 years of the surgery, reveals no tumor. Conclusion: Hemangioblastoma is a rare tumor, especially in its supratentorial location. However, this case reflects the importance of including HBL as a differential diagnostic allowing a better therapeutic planning, ensuring better prognosis and facilitating their treatment.

Published in	International Journal of Neurosurgery (Volume 5, Issue 1)
DOI	10.11648/j.ijn.20210501.11
Page(s)	1-3
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Hemangioblastoma, Supratentorial, Von Hippel Lindau, Brain Tumor

References

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[2]	Yang B, Luan S, Cao X, Bao W. Supratentorial hemangioblastoma. Neurosciences. 2011; 16 (2): 150-152. doi: 10.1007/BF01402059.
[3]	Pandey S, Sharma V, Pandey D, Kumar V, Kumar M. Supratentorial haemangioblastoma without von Hippel-Lindau syndrome in an adult: A rare tumor with review of literature. Asian J Neurosurg. 2016; 11 (1): 8-14. doi: 10.4103/1793-5482.165782.
[4]	Lee CY, Chen SJ, Yang B, et al. Supratentorial haemangioblastoma without von Hippel-Lindau syndrome in an adult: A rare tumor with review of literature. Korean J Pathol. 2014; 6 (2): 462-465. doi: 10.1007/BF01402059.
[5]	Natural History of Supratentorial Hemangioblastomas in von Hippel-Lindau Disease. Neurosurgery. 2010; 67 (3): 577-587. doi: 10.1227/01.NEU.0000374846.86409.A7.
[6]	Wenting J, Ogawa Y, Ito J, Tominaga T. Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein. J Neurol Surgery, Part A Cent Eur Neurosurg. 2017; 78 (3): 296-301. doi: 10.1055/s-0036-1582016.
[7]	Franco A, Pytel P, Lukas R V., Chennamaneni R, Collins JM. CNS hemangioblastomatosis in a patient without von Hippel-Lindau disease. CNS Oncol. 2017; 6 (2): 101-105. doi: 10.2217/cns-2016-0027.
[8]	Mills SA, Oh MC, Rutkowski MJ, Sughrue ME, Barani IJ, Parsa AT. Supratentorial hemangioblastoma: Clinical features, prognosis, and predictive value of location for von HippelLindau disease. Neuro Oncol. 2012; 14 (8): 1097-1104. doi: 10.1093/neuonc/nos133.
[9]	She DJ, Xing Z, Liu Y, Cao DR. Supratentorial hemangioblastomas: Three case reports and review of the literature. Clin Neuroradiol. 2013; 23 (3): 243-247. doi: 10.1007/s00062-012-0183-9.
[10]	Rocha L, Noronha C, Taipa R, Reis J, Gomes M, Carvalho E. Supratentorial hemangioblastomas in von Hippel–Lindau wild-type patients–case series and literature review. Int J Neurosci. 2018; 128 (3): 295-303. doi: 10.1080/00207454.2017.1385613.
[11]	Lonser RR, Butman JA, Huntoon K, et al. Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 2014; 120 (5): 1055-1062. doi: 10.3171/2014.1.JNS131431.
[12]	Report AC. A Case Report and Review of the Literature. 2003: 1-3.
[13]	Lee CY, Chen SJ. Supratentorial leptomeningeal hemangioblastoma resection after tumor embolization. Formos J Surg. 2016; 49 (2): 78-81. doi: 10.1016/j.fjs.2016.02.002.

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APA Style

Jose Carlos Rodrigues Junior, Vithor Ely Bortholin da Silva, Roberto Bezerra Vital, Renan Luiz Lapate, Marcelo Ferraz Campos. (2021). Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review. International Journal of Neurosurgery, 5(1), 1-3. https://doi.org/10.11648/j.ijn.20210501.11

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ACS Style

Jose Carlos Rodrigues Junior; Vithor Ely Bortholin da Silva; Roberto Bezerra Vital; Renan Luiz Lapate; Marcelo Ferraz Campos. Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review. Int. J. Neurosurg. 2021, 5(1), 1-3. doi: 10.11648/j.ijn.20210501.11

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AMA Style

Jose Carlos Rodrigues Junior, Vithor Ely Bortholin da Silva, Roberto Bezerra Vital, Renan Luiz Lapate, Marcelo Ferraz Campos. Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review. Int J Neurosurg. 2021;5(1):1-3. doi: 10.11648/j.ijn.20210501.11

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@article{10.11648/j.ijn.20210501.11,
  author = {Jose Carlos Rodrigues Junior and Vithor Ely Bortholin da Silva and Roberto Bezerra Vital and Renan Luiz Lapate and Marcelo Ferraz Campos},
  title = {Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review},
  journal = {International Journal of Neurosurgery},
  volume = {5},
  number = {1},
  pages = {1-3},
  doi = {10.11648/j.ijn.20210501.11},
  url = {https://doi.org/10.11648/j.ijn.20210501.11},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijn.20210501.11},
  abstract = {Background and Importance: Hemangioblastomas (HBL) are tumors constantly present in the neurosurgeon's daily life, affecting mainly the posterior fossa region. In patients with von Hippel-Lindau, their presence is even more striking. However, the authors, through a report of a case with the literature review, show that hemangioblastomas should also be part of the range of differential diagnoses of supratentorial lesions even in patients not carrying the genetic mutation. Clinical Presentation: A 45-year-old male patient has a 2 weeks history of headache associated with nauseas and vertigo. In the last two weeks the pain was being progressively more frequently with increase in intensity and duration. At examination he was oriented, with face of pain, muscle examination was normal as all the neurological examination. The patient was submitted to a Magnetic Resonance (MR) of the brain that showed an expansive lesion in the brain located in the left frontal lobe, adjacent to the medial line with moderated adjacent edema and with gadolinium injection the tumor was heterogeneous almost solid with cystic parts and including adjacent impregnation of the dura of the midline. Surgical treatment was done. The lesion was completed resected including the dura of the convexity that was invaded by the tumor. Postoperative period was uneventful, with resolution of the headache and no deficits. Patient was discharged 3 days after surgery. Pathology reveals the lesion was a hemangioblastoma. Patient made genetic exams and confirms that didn’t have Von Hippel Lindau disease. MR of the brain, almost 3 years of the surgery, reveals no tumor. Conclusion: Hemangioblastoma is a rare tumor, especially in its supratentorial location. However, this case reflects the importance of including HBL as a differential diagnostic allowing a better therapeutic planning, ensuring better prognosis and facilitating their treatment.},
 year = {2021}
}

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TY - JOUR
T1 - Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review
AU - Jose Carlos Rodrigues Junior
AU - Vithor Ely Bortholin da Silva
AU - Roberto Bezerra Vital
AU - Renan Luiz Lapate
AU - Marcelo Ferraz Campos
Y1 - 2021/01/04
PY - 2021
N1 - https://doi.org/10.11648/j.ijn.20210501.11
DO - 10.11648/j.ijn.20210501.11
T2 - International Journal of Neurosurgery
JF - International Journal of Neurosurgery
JO - International Journal of Neurosurgery
SP - 1
EP - 3
PB - Science Publishing Group
SN - 2640-1959
UR - https://doi.org/10.11648/j.ijn.20210501.11
AB - Background and Importance: Hemangioblastomas (HBL) are tumors constantly present in the neurosurgeon's daily life, affecting mainly the posterior fossa region. In patients with von Hippel-Lindau, their presence is even more striking. However, the authors, through a report of a case with the literature review, show that hemangioblastomas should also be part of the range of differential diagnoses of supratentorial lesions even in patients not carrying the genetic mutation. Clinical Presentation: A 45-year-old male patient has a 2 weeks history of headache associated with nauseas and vertigo. In the last two weeks the pain was being progressively more frequently with increase in intensity and duration. At examination he was oriented, with face of pain, muscle examination was normal as all the neurological examination. The patient was submitted to a Magnetic Resonance (MR) of the brain that showed an expansive lesion in the brain located in the left frontal lobe, adjacent to the medial line with moderated adjacent edema and with gadolinium injection the tumor was heterogeneous almost solid with cystic parts and including adjacent impregnation of the dura of the midline. Surgical treatment was done. The lesion was completed resected including the dura of the convexity that was invaded by the tumor. Postoperative period was uneventful, with resolution of the headache and no deficits. Patient was discharged 3 days after surgery. Pathology reveals the lesion was a hemangioblastoma. Patient made genetic exams and confirms that didn’t have Von Hippel Lindau disease. MR of the brain, almost 3 years of the surgery, reveals no tumor. Conclusion: Hemangioblastoma is a rare tumor, especially in its supratentorial location. However, this case reflects the importance of including HBL as a differential diagnostic allowing a better therapeutic planning, ensuring better prognosis and facilitating their treatment.
VL - 5
IS - 1
ER -

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Author Information

Jose Carlos Rodrigues Junior

Department of Neurosurgery, Heliópolis Hospital, S?o Paulo, Brazil; Department of Neurosurgery, Assun??o Hospital, S?o Paulo, Brazil
Vithor Ely Bortholin da Silva

Department of Neurosurgery, Heliópolis Hospital, S?o Paulo, Brazil
Roberto Bezerra Vital

Department of Neurosurgery, Assun??o Hospital, S?o Paulo, Brazil
Renan Luiz Lapate

Department of Neurosurgery, Assun??o Hospital, S?o Paulo, Brazil
Marcelo Ferraz Campos

Department of Neurosurgery, Heliópolis Hospital, S?o Paulo, Brazil

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APA Style

Jose Carlos Rodrigues Junior, Vithor Ely Bortholin da Silva, Roberto Bezerra Vital, Renan Luiz Lapate, Marcelo Ferraz Campos. (2021). Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review. International Journal of Neurosurgery, 5(1), 1-3. https://doi.org/10.11648/j.ijn.20210501.11

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ACS Style

Jose Carlos Rodrigues Junior; Vithor Ely Bortholin da Silva; Roberto Bezerra Vital; Renan Luiz Lapate; Marcelo Ferraz Campos. Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review. Int. J. Neurosurg. 2021, 5(1), 1-3. doi: 10.11648/j.ijn.20210501.11

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AMA Style

Jose Carlos Rodrigues Junior, Vithor Ely Bortholin da Silva, Roberto Bezerra Vital, Renan Luiz Lapate, Marcelo Ferraz Campos. Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review. Int J Neurosurg. 2021;5(1):1-3. doi: 10.11648/j.ijn.20210501.11

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@article{10.11648/j.ijn.20210501.11,
  author = {Jose Carlos Rodrigues Junior and Vithor Ely Bortholin da Silva and Roberto Bezerra Vital and Renan Luiz Lapate and Marcelo Ferraz Campos},
  title = {Supratentorial Hemangioblastomas Without Von - Hippel Lindau Disease – Case Report and Review},
  journal = {International Journal of Neurosurgery},
  volume = {5},
  number = {1},
  pages = {1-3},
  doi = {10.11648/j.ijn.20210501.11},
  url = {https://doi.org/10.11648/j.ijn.20210501.11},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ijn.20210501.11},
  abstract = {Background and Importance: Hemangioblastomas (HBL) are tumors constantly present in the neurosurgeon's daily life, affecting mainly the posterior fossa region. In patients with von Hippel-Lindau, their presence is even more striking. However, the authors, through a report of a case with the literature review, show that hemangioblastomas should also be part of the range of differential diagnoses of supratentorial lesions even in patients not carrying the genetic mutation. Clinical Presentation: A 45-year-old male patient has a 2 weeks history of headache associated with nauseas and vertigo. In the last two weeks the pain was being progressively more frequently with increase in intensity and duration. At examination he was oriented, with face of pain, muscle examination was normal as all the neurological examination. The patient was submitted to a Magnetic Resonance (MR) of the brain that showed an expansive lesion in the brain located in the left frontal lobe, adjacent to the medial line with moderated adjacent edema and with gadolinium injection the tumor was heterogeneous almost solid with cystic parts and including adjacent impregnation of the dura of the midline. Surgical treatment was done. The lesion was completed resected including the dura of the convexity that was invaded by the tumor. Postoperative period was uneventful, with resolution of the headache and no deficits. Patient was discharged 3 days after surgery. Pathology reveals the lesion was a hemangioblastoma. Patient made genetic exams and confirms that didn’t have Von Hippel Lindau disease. MR of the brain, almost 3 years of the surgery, reveals no tumor. Conclusion: Hemangioblastoma is a rare tumor, especially in its supratentorial location. However, this case reflects the importance of including HBL as a differential diagnostic allowing a better therapeutic planning, ensuring better prognosis and facilitating their treatment.},
 year = {2021}
}

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