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J Neurol Surg A Cent Eur Neurosurg
DOI: 10.1055/a-2521-3080
Case Report

Rapid Clinical Deterioration of a Patient with a Posterior Fossa Hemangioblastoma and Extensive Cerebellar Ischemia: Report of a Unique Case

Adrian Liebert
1   Department of Neurosurgery, Klinikum Nürnberg, Paracelsus Medical University, Nuremberg, Bavaria, Germany
,
Karl-Michael Schebesch
1   Department of Neurosurgery, Klinikum Nürnberg, Paracelsus Medical University, Nuremberg, Bavaria, Germany
,
Cristiane Blechschmidt
2   Institute for Pathology, Klinikum Nürnberg, Paracelsus Medical University, Nuremberg, Bavaria, Germany
,
Thomas Eibl
1   Department of Neurosurgery, Klinikum Nürnberg, Paracelsus Medical University, Nuremberg, Bavaria, Germany
,
Leonard Ritter
1   Department of Neurosurgery, Klinikum Nürnberg, Paracelsus Medical University, Nuremberg, Bavaria, Germany
› Author Affiliations Funding None.
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Abstract

Introduction

Hemangioblastomas are rare, benign, highly vascularized tumors, which among other locations arise in the central nervous system. Due to the tumor's dense vascularity, bleeding and interference with the surrounding brain tissue and vasculature have been reported. Rapid neurological deterioration due to hemorrhage from a hemangioblastoma, especially in spinal locations, has been reported.

Case Description

Rapid clinical deterioration occurred in a 47-year-old male patient with a cerebellar hemangioblastoma and delayed extensive cerebellar ischemia, consecutively. Initial cranial magnetic resonance imaging revealed the tumor with small ischemic areas in the left cerebellar hemisphere. A couple of days later, consciousness dropped significantly and immediate computed tomography revealed extensive ischemia of the left cerebellar hemisphere. Emergency suboccipital decompressive craniectomy and tumor resection were performed. The patient recovered and was discharged to neurological rehabilitation a couple of weeks later.

Conclusion

Despite the benign character of hemangioblastomas, life-threatening rapid deterioration due to cerebellar ischemia can occur as reported in this case.

Keywords

hemangioblastoma - ischemia - emergency surgery

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.




Publication History

Received: 11 September 2024

Accepted: 20 January 2025

Article published online:
15 May 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

  • References

  • 1 Andrews DW. Our current knowledge of hemangioblastomas and treatment. World Neurosurg 2011; 75 (01) 45-46
    PubMedSearch in Google Scholar
  • 2 Mills SA, Oh MC, Rutkowski MJ, Sughrue ME, Barani IJ, Parsa AT. Supratentorial hemangioblastoma: clinical features, prognosis, and predictive value of location for von Hippel-Lindau disease. Neuro-oncol 2012; 14 (08) 1097-1104
    CrossrefPubMedSearch in Google Scholar
  • 3 Louis DN, Perry A, Wesseling P. et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro-oncol 2021; 23 (08) 1231-1251
    CrossrefPubMedSearch in Google Scholar
  • 4 Wanebo JE, Lonser RR, Glenn GM, Oldfield EH. The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 2003; 98 (01) 82-94
    CrossrefPubMedSearch in Google Scholar
  • 5 Jagannathan J, Lonser RR, Smith R, DeVroom HL, Oldfield EH. Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 2008; 108 (02) 210-222
    CrossrefPubMedSearch in Google Scholar
  • 6 de San Pedro JR, Rodríguez FA, Níguez BF. et al. Massive hemorrhage in hemangioblastomas literature review. Neurosurg Rev 2010; 33 (01) 11-26
    CrossrefPubMedSearch in Google Scholar
  • 7 Chen X, Wang M, Tang Y, Xie B, Nie X, Cai S. Von Hipple-Lindau disease complicated with central retinal vein occlusion: a case report. BMC Ophthalmol 2022; 22 (01) 440
    PubMedSearch in Google Scholar
  • 8 Bründl E, Schödel P, Ullrich OW, Brawanski A, Schebesch KM. Surgical resection of sporadic and hereditary hemangioblastoma: our 10-year experience and a literature review. Surg Neurol Int 2014; 5: 138
    CrossrefPubMedSearch in Google Scholar
  • 9 Gläsker S, Van Velthoven V. Risk of hemorrhage in hemangioblastomas of the central nervous system. Neurosurgery 2005; 57 (01) 71-76 , discussion 71–76
    CrossrefPubMedSearch in Google Scholar
  • 10 Gluf WM, Dailey AT. Hemorrhagic intramedullary hemangioblastoma of the cervical spinal cord presenting with acute-onset quadriparesis: case report and review of the literature. J Spinal Cord Med 2014; 37 (06) 791-794
    PubMedSearch in Google Scholar
  • 11 Koda M, Mannoji C, Itabashi T. et al. Intramedullary hemorrhage caused by spinal cord hemangioblastoma: a case report. BMC Res Notes 2014; 7 (01) 823
    PubMedSearch in Google Scholar
  • 12 Ammerman JM, Lonser RR, Dambrosia J, Butman JA, Oldfield EH. Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: implications for treatment. J Neurosurg 2006; 105 (02) 248-255
    PubMedSearch in Google Scholar
  • 13 Kuharic M, Jankovic D, Splavski B, Boop FA, Arnautovic KI. Hemangioblastomas of the posterior cranial fossa in adults: demographics, clinical, morphologic, pathologic, surgical features, and outcomes. a systematic review. World Neurosurg 2018; 110: e1049-e1062
    CrossrefPubMedSearch in Google Scholar
  • 14 Sun Z, Yuan D, Sun Y, Yan P, Zuo H. Surgical resection of cerebellar hemangioblastoma with enhanced wall thickness: a report of two cases. Oncol Lett 2015; 9 (04) 1597-1599
    PubMedSearch in Google Scholar
  • 15 Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery 2001; 48 (01) 55-62 , discussion 62–63
    PubMedSearch in Google Scholar
  • 16 Tomasello F, La Torre D. Predictors of outcome in surgery for sporadic cerebellar hemangioblastomas. World Neurosurg 2014; 82 (05) 621-622
    PubMedSearch in Google Scholar
  • 17 Pan J, Jabarkheel R, Huang Y, Ho A, Chang SD. Stereotactic radiosurgery for central nervous system hemangioblastoma: systematic review and meta-analysis. J Neurooncol 2018; 137 (01) 11-22
    PubMedSearch in Google Scholar
  • 18 Shepherd STC, Drake WM, Turajlic S. The road to systemic therapy in von Hippel-Lindau (VHL) disease: are we there yet?. Eur J Cancer 2023; 182: 15-22
    PubMedSearch in Google Scholar
  • 19 Hartkamp NS, De Cocker LJ, Helle M. et al. In vivo visualization of the PICA perfusion territory with super-selective pseudo-continuous arterial spin labeling MRI. Neuroimage 2013; 83: 58-65
    PubMedSearch in Google Scholar
  • 20 De Cocker LJL, Lövblad KO, Hendrikse J. MRI of cerebellar infarction. Eur Neurol 2017; 77 (3-4): 137-146
    CrossrefPubMedSearch in Google Scholar
  • 21 Kumral E, Kisabay A, Ataç C, Çallı C, Yünten N. Spectrum of the posterior inferior cerebellar artery territory infarcts. Clinical-diffusion-weighted imaging correlates. Cerebrovasc Dis 2005; 20 (05) 370-380
    PubMedSearch in Google Scholar
  • 22 Willinsky R, Terbrugge K, Montanera W, Mikulis D, Wallace MC. Venous congestion: an MR finding in dural arteriovenous malformations with cortical venous drainage. AJNR Am J Neuroradiol 1994; 15 (08) 1501-1507
    PubMedSearch in Google Scholar
  • 23 Shimizu A, Ishikawa T, Yamaguchi K. et al. Brainstem venous congestion caused by perimedullary drainage in anterior cranial fossa dural arteriovenous fistula. World Neurosurg 2019; 127: 503-508
    PubMedSearch in Google Scholar
  • 24 Rachinger J, Buslei R, Prell J, Strauss C. Solid haemangioblastomas of the CNS: a review of 17 consecutive cases. Neurosurg Rev 2009; 32 (01) 37-47 , discussion 47–48
    CrossrefPubMedSearch in Google Scholar
  • 25 Hojo M, Arakawa Y, Funaki T. et al. Usefulness of tumor blood flow imaging by intraoperative indocyanine green videoangiography in hemangioblastoma surgery. World Neurosurg 2014; 82 (3-4): e495-e501
    CrossrefPubMedSearch in Google Scholar
  • 26 Fiehler J, Illies T, Piening M. et al. Territorial and microvascular perfusion impairment in brain arteriovenous malformations. AJNR Am J Neuroradiol 2009; 30 (02) 356-361
    PubMedSearch in Google Scholar
  • 27 Ju C, Wright CH, Wright J, Duan Y, Bambakidis NC. Subarachnoid hemorrhage associated with intratumoral aneurysm rupture within a posterior fossa hemangioblastoma: the importance of continued surveillance for cerebral vasospasm. Cureus 2017; 9 (08) e1606
    PubMedSearch in Google Scholar
  • 28 Lee Y, Cheng SM, Hwang DY, Chiu YL, Chou YH. Polycythemia secondary to renal hemangioblastoma: a case report and literature review. Int J Surg Pathol 2024; 32 (01) 140-144
    PubMedSearch in Google Scholar