Skip to main content

Advertisement

Log in

Massive hemorrhage in hemangioblastomas

Literature review

  • Review
  • Published:
Neurosurgical Review Aims and scope Submit manuscript

Abstract

Massive hemorrhage is a very uncommon event among hemangioblastomas. Forty-four cases have been reported before this review. Thorough analysis of all reported cases on literature was accomplished. The majority presented as parenchymatous or subarachnoid bleedings. Subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances. Ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment. To our knowledge, the illustrative case is the first one of pure tetraventricular hemorrhage from a hemangioblastoma. When hemangioblastoma bleeding occurs, it is usually the very first clinical presentation of a previously undetected tumor. Solid type, large size, and spinal–radicular locations are more frequently related to hemorrhage. Hemorrhage following hemangioblastoma embolization and the association of this tumor with other bleeding lesions, such as arteriovenous malformations and aneurysms, is also discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Adegbite AB, Rozdilsky B, Varughese G (1983) Supratentorial capillary hemangioblastoma presenting with fatal spontaneous intracerebral hemorrhage. Neurosurgery 12:327–330

    Article  CAS  PubMed  Google Scholar 

  2. Bachmann K, Markwalder R, Seiler RW (1978) Supratentorial haemangioblastoma. Case report. Acta Neurochir (Wien) 44:173–177

    Article  CAS  Google Scholar 

  3. Baker KB, Moran CJ, Wippold FJ 2nd, Smirniotopoulos JG, Rodriguez FJ, Meyers SP et al (2000) MR imaging of spinal hemangioblastoma. AJR Am J Roentgenol 174:377–382

    CAS  PubMed  Google Scholar 

  4. Berlis A, Schumacher M, Spreer J, Neumann HP, van Velthoven V (2003) Subarachnoid haemorrhage due to cervical spinal cord haemangioblastomas in a patient with von Hippel-Lindau disease. Acta Neurochir (Wien) 145:1009–1013

    Article  CAS  Google Scholar 

  5. Bertalanffy H, Mennel HD, Benes L, Riegel T, Aboul-Enein H (2003) Isolated paramedullary hemangioblastoma originating from the first cervical nerve root: case report. Spine 28:E191–E193

    Article  PubMed  Google Scholar 

  6. Brown RD Jr, Wiebers DO, Torner JC, O'Fallon WM (1996) Frequency of intracranial hemorrhage as a presenting symptom and subtype analysis: a population-based study of intracranial vascular malformations in Olmsted Country, Minnesota. J Neurosurg 85:29–32

    Article  PubMed  Google Scholar 

  7. Browne TR, Adams RD, Robertson GH (1976) Hemangioblastoma of the spinal cord. Review and report of five cases. Arch Neurol 33:435–441

    CAS  PubMed  Google Scholar 

  8. Catapano D, Muscarella LA, Guarnieri V, Zelante L, D'Angelo VA, D'Agruma L (2005) Hemangioblastomas of central nervous system: molecular genetic analysis and clinical management. Neurosurgery 56:1215–1221

    Article  PubMed  Google Scholar 

  9. Cerejo A, Vaz R, Feyo PB, Cruz C (1990) Spinal cord hemangioblastoma with subarachnoid hemorrhage. Neurosurgery 27:991–993

    Article  CAS  PubMed  Google Scholar 

  10. Cervoni L, Franco C, Celli P, Fortuna A (1995) Spinal tumors and subarachnoid hemorrhage: pathogenetic and diagnostic aspects in 5 cases. Neurosurg Rev 18:159–162

    Article  CAS  PubMed  Google Scholar 

  11. Choyke PL, Glenn GM, Walther MM, Patronas NJ, Linehan WM, Zbar B (1995) von Hippel-Lindau disease: genetic, clinical, and imaging features. Radiology 194:629–642 Erratum in: Radiology. 196(2):582

    CAS  PubMed  Google Scholar 

  12. Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D (2001) Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery 48:55–62

    Article  CAS  PubMed  Google Scholar 

  13. Cornelius JF, Saint-Maurice JP, Bresson D, George B, Houdart E (2007) Hemorrhage after particle embolization of hemangioblastomas: comparison of outcomes in spinal and cerebellar lesions. J Neurosurg 106:994–998

    Article  PubMed  Google Scholar 

  14. Dijindjian M, Djindjian R, Houdart R, Hurth M (1978) Subarachnoid hemorrhage due to intraspinal tumors. Surg Neurol 9:223–229

    CAS  PubMed  Google Scholar 

  15. Eskridge JM, McAuliffe W, Harris B, Kim DK, Scott J, Winn HR (1996) Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol 17:525–531

    CAS  PubMed  Google Scholar 

  16. Fleetwood IG, Hamilton MG (2004) Hemorrhagic disease: arteriovascular malformations. In: Winn HR (ed) Youmans neurological surgery, vol 2, 5th edn. Saunders, Philadelphia, pp 2137–2158

    Google Scholar 

  17. Flemming KD, Brown RD Jr (2004) Natural history of intracranial vascular malformations. In: Winn HR (ed) Youmans neurological surgery, vol 2, 5th edn. Saunders, Philadelphia, pp 2159–2183

    Google Scholar 

  18. Friedrich H, Hänsel-Friedrich G, Zeumer H (1990) Intramedullary vascular lesions in the high cervical region: transoral and dorsal surgical approach. Two case reports. Neurosurg Rev 13:65–71

    Article  CAS  PubMed  Google Scholar 

  19. Fujii H, Higashi S, Hashimoto M, Shouin K, Hayase H, Kimura M et al (1987) Hemangioblastoma presenting with fourth ventricular bleeding. Case report. Neurol Med Chir (Tokyo) 27:545–549 Japanese

    Article  CAS  Google Scholar 

  20. Fukuma S, Takemoto S, Kakita K, Ohmachi J, Sasaki R (1977) Cerebellar hemorrhage caused by hemangioblastoma: a case report. Presented at the 6th Proceeding of Japanese Conference on Surgery for Cerebral Stroke. Tokyo, pp 185–188, Japanese

  21. Gläsker S (2005) Central nervous system manifestations in VHL: genetics, pathology and clinical phenotypic features. Fam Cancer 4:37–42

    Article  PubMed  Google Scholar 

  22. Gläsker S, Bender BU, Apel TW, Natt E, van Velthoven V, Scheremet R et al (1999) The impact of molecular genetic analysis of the VHL gene in patients with haemangioblastomas of the central nervous system. J Neurol Neurosurg Psychiatry 67:758–762

    Article  PubMed  Google Scholar 

  23. Gläsker S, Berlis A, Pagenstecher A, Vougioukas VI, Van Velthoven V (2005) Characterization of hemangioblastomas of spinal nerves. Neurosurgery 56:503–509

    Article  PubMed  Google Scholar 

  24. Gläsker S, Van Velthoven V (2005) Risk of hemorrhage in hemangioblastomas of the central nervous system. Neurosurgery 57:71–76

    Article  PubMed  Google Scholar 

  25. Guzman R, Grady MS (1999) An intracranial aneurysm on the feeding artery of a cerebellar hemangioblastoma. Case report. J Neurosurg 91:136–138

    Article  CAS  PubMed  Google Scholar 

  26. Hashimoto K, Nozaki K, Oda Y, Kikuchi H (1995) Cerebellar hemangioblastoma with intracystic hemorrhage—case report. Neurol Med Chir (Tokyo) 35:458–461

    Article  CAS  Google Scholar 

  27. Hatva E, Böhling T, Jääskeläinen J, Persico MG, Haltia M, Alitalo K (1996) Vascular growth factors and receptors in capillary hemangioblastomas and hemangiopericytomas. Am J Pathol 148:763–775

    CAS  PubMed  Google Scholar 

  28. Ho KL (1985) Ultrastructure of cerebellar capillary hemangioblastoma. III. Crystalloid bodies in endothelial cells. Acta Neuropathol 66:117–126

    Article  CAS  PubMed  Google Scholar 

  29. Hoff DJ, Tampieri D, Just N (1993) Imaging of spinal cord hemangioblastomas. Can Assoc Radiol J 44:377–383

    CAS  PubMed  Google Scholar 

  30. Hwang SI, Jeon SS, Cho KK, Park CK, Sung WH, Lee SW et al (1993) A case of a supratentorial hemangioblastoma associated with intraparenchymatous hemorrhage. J Korean Neurosurg Soc 22:571–576 Korean

    Google Scholar 

  31. Irie K, Kuyama H, Nagao S (1998) Spinal cord hemangioblastoma presenting with subarachnoid hemorrhage. Neurol Med Chir (Tokyo) 38:355–358

    Article  CAS  Google Scholar 

  32. Ishikawa E, Matsumura A, Matsumaru Y, Anno I, Nakamura K, Shioyama Y et al (2002) Intratumoral hemorrhage due to hemangioblastoma arising from a cervical nerve root—a case report. J Clin Neurosci 9:713–716

    Article  PubMed  Google Scholar 

  33. Jagannathan J, Lonser RR, Smith R, DeVroom HL, Oldfield EH (2008) Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 108:210–222

    Article  PubMed  Google Scholar 

  34. Kikuchi K, Kowada M, Sasaki J, Yanagida N (1994) Cerebellar hemangioblastoma associated with fatal intratumoral hemorrhage: report of an autopsied case. No Shinkei Geka 22:593–597 Japanese

    CAS  PubMed  Google Scholar 

  35. Kim JM, Cheong JH, Bak KH, Kim CH, Park DW, Oh YH (2006) Congenital supratentorial hemangioblastoma as an unusual cause of simultaneous supra- and infratentorial intracranial hemorrhage: case report. J Neurooncol 77:59–63

    Article  PubMed  Google Scholar 

  36. Kim JE, Oh CW, Choi KS, Han DH (1996) Pontine hemangioblastoma presenting with pontine hemorrhage. Case report. J Korean Neurosurg Soc 25:2071–2075 Korean

    Google Scholar 

  37. Kormos RL, Tucker WS, Bilbao JM, Gladstone RM, Bass AG (1980) Subarachnoid hemorrhage due to a spinal cord hemangioblastoma: case report. Neurosurgery 6:657–660

    Article  CAS  PubMed  Google Scholar 

  38. Krishnan KG, Schackert G (2006) Outcomes of surgical resection of large solitary hemangioblastomas of the craniocervical junction with limitations in preoperative angiographic intervention: report of three cases. Zentralbl Neurochir 67:137–143

    Article  CAS  PubMed  Google Scholar 

  39. Lee JY, Cho BM, Oh SM, Park SH (2007) Delayed diagnosis of cerebellar hemangioblastoma after intracerebellar hemorrhage. Surg Neurol 67:419–421

    Article  PubMed  Google Scholar 

  40. Lee SR, Sanches J, Mark AS, Dillon WP, Norman D, Newton TH (1989) Posterior fossa hemangioblastomas: MR imaging. Radiology 171:463–468

    CAS  PubMed  Google Scholar 

  41. Lymaye U, Siddhartha W, Shrivastava M, Parmar H (2000) Posterior fossa hypervascular tumor embolisation. An unusual complication. Riv Neuroradiol 13:593–598

    Google Scholar 

  42. Malis LI (2002) A traumatic bloodless removal of intramedullary hemangioblastomas of the spinal cord. J Neurosurg Spine 97:1–6

    Article  Google Scholar 

  43. Matsumura A, Maki Y, Munekata K, Kobayashi E (1985) Intracerebellar hemorrhage due to cerebellar hemangioblastoma. Surg Neurol 24:227–230

    Article  CAS  PubMed  Google Scholar 

  44. Matsunaga S, Shuto T, Inomori S, Fujino H, Yamamoto I (2007) Gamma knife radiosurgery for intracranial haemangioblastomas. Acta Neurochir (Wien) 149:1007–1013

    Article  CAS  Google Scholar 

  45. Melmon KL, Rosen SW (1965) Lindau’s disease: review of the literature and study of a large kindred. Am J Med 36:595–617

    Article  Google Scholar 

  46. Menovsky T, André Grotenhuis J, Bartels RH (2002) Aneurysm of the anterior inferior cerebellar artery (AICA) associated with high-flow lesion: report of two cases and review of literature. J Clin Neurosci 9:207–211

    Article  PubMed  Google Scholar 

  47. Minami M, Hanakita J, Suwa H, Suzui H, Fujita K, Nakamura T (1998) Cervical hemangioblastoma with a past history of subarachnoid hemorrhage. Surg Neurol 49:278–281

    Article  CAS  PubMed  Google Scholar 

  48. Montano N, Doglietto F, Pedicelli A, Albanese A, Lauretti L, Pallini R, Lauriola L, Fernandez E, Maira G (2008) Embolization of hemangioblastomas. J Neurosurg 108:1063–1064 author reply 1064–1065 (Letter)

    Article  PubMed  Google Scholar 

  49. Murai Y, Kobayashi S, Tateyama K, Teramoto A (2006) Persistent primitive trigeminal artery aneurysm associated with cerebellar hemangioblastoma. Case report. Neurol Med Chir (Tokyo) 46:143–146

    Article  Google Scholar 

  50. Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P (1989) Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg 70:24–30

    Article  CAS  PubMed  Google Scholar 

  51. Niemelä M, Lim YJ, Söderman M, Jääskeläinen J, Lindquist C (1996) Gamma knife radiosurgery in 11 hemangioblastomas. J Neurosurg 85:591–596

    Article  PubMed  Google Scholar 

  52. Nishio S, Koga M, Ohta M, Nagata S, Ohgami S (1981) Two autopsy cases of brain tumor manifested by subarachnoid hemorrhage. Shinkei Naika 14:167–171 Japanese

    Google Scholar 

  53. Orita T, Abiko S, Aoki H, Hatano M (1985) Hemangioblastoma with massive bleeding. Case report. Neurol Med Chir (Tokyo) 25:398–403 Japanese

    Article  CAS  Google Scholar 

  54. Rainov NG, Burkert WL (1995) Urokinase infusion for severe intraventricular haemorrhage. Acta Neurochir (Wien) 134:55–59

    Article  CAS  Google Scholar 

  55. Rothbart D, Awad IA, Lee J, Kim J, Harbaugh R, Criscuolo GR (1996) Expression of angiogenic factors and structural proteins in central nervous system vascular malformations. Neurosurgery 38:915–924

    Article  CAS  PubMed  Google Scholar 

  56. Russel DS, Rubinstein LJ (1977) Pathology of tumours of the nervous system, 4th edn. Edward Arnold, London, pp 116–127

    Google Scholar 

  57. Ryang YM, Oertel MF, Thron A, Gilsbach J, Rohde V (2007) Rare intramedullary hemorrhage of a brainstem hemangioblastoma. Zentralbl Neurochir 68:29–33

    Article  PubMed  Google Scholar 

  58. Ryu KY, Cho JH, Lee SL, Kang DG, Kim SC (1997) Congenital cystic supratentorial hemangioblastoma associated with intracystic hemorrhage: case report. J Korean Neurosurg Soc 26:879–882 Korean

    Google Scholar 

  59. Shin BC, Park K, Min BK, Suk JS, Choi DY (1989) Cerebellar hemangioblastoma. Hemorrhage as an initial presentation. J Korean Neurosurg Soc 18:482–485 Korean

    Google Scholar 

  60. Silver ML, Hennigar G (1952) Cerebellar hemangioma (hemangioblastoma). A clinicopathological review of 40 cases. J Neurosurg 9:484–494

    Article  CAS  PubMed  Google Scholar 

  61. Skirgaudas M, Awad IA, Kim J, Rothbart D, Criscuolo G (1996) Expression of angiogenesis factors and selected vascular wall matrix proteins in intracranial saccular aneurysms. Neurosurgery 39:537–545

    Article  CAS  PubMed  Google Scholar 

  62. Slater A, Moore NR, Huson SM (2003) The natural history of cerebellar hemangioblastomas in von Hippel-Lindau disease. AJNR Am J Neuroradiol 24:1570–1574

    PubMed  Google Scholar 

  63. Surawicz TS, McCarthy BJ, Kupelian V, Jukich PJ, Bruner JM, Davis FG (1999) Descriptive epidemiology of primary brain and CNS tumors: results from the Central Brain Tumor Registry of the United States, 1990–1994. Neuro Oncol 1:14–25

    CAS  PubMed  Google Scholar 

  64. Tago M, Terahara A, Shin M, Maruyama K, Kurita H, Nakagawa K, Ohtomo K (2005) Gamma knife surgery for hemangioblastomas. J Neurosurg 102(Suppl):171–174

    Article  PubMed  Google Scholar 

  65. Tampieri D, Leblanc R, TerBrugge K (1993) Preoperative embolization of brain and spinal hemangioblastomas. Neurosurgery 33:502–505

    Article  CAS  PubMed  Google Scholar 

  66. Ueno K, Mabuchi S, Echizenya K, Isu T, Goto S (1977) Incidentally-discovered aneurysm—a report of eight cases. No Shinkei Geka 5:183–188. Japanese

    CAS  PubMed  Google Scholar 

  67. Unemori EN, Ferrara N, Bauer EA, Amento EP (1992) Vascular endothelial growth factor induces interstitial collagenase expression in human endothelial cells. J Cell Physiol 153:557–562

    Article  CAS  PubMed  Google Scholar 

  68. Van Hille PT, Abbott RJ, Cameron MM, Holland IM, Loizou LA (1987) Recurrent spontaneous subarachnoid haemorrhage due to spinal haemangioblastoma. J Neurol Neurosurg Psychiatry 50:639–640 Letter

    Article  PubMed  Google Scholar 

  69. Vates GE, Berger MS (2004) Hemangioblastomas of the central nervous system. In: Winn HR (ed) Youmans neurological surgery, vol 1, 5th edn. Saunders, Philadelphia, pp 1053–1066

    Google Scholar 

  70. Wakai S, Inoh S, Ueda Y, Nagai M (1984) Hemangioblastoma presenting with intraparenchymatous hemorrhage. J Neurosurg 61:956–960

    Article  CAS  PubMed  Google Scholar 

  71. Wanebo JE, Lonser RR, Glenn GM, Oldfield EH (2003) The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg 98:82–94

    Article  PubMed  Google Scholar 

  72. Wisoff HS, Suzuki Y, Llena JF, Fine DI (1978) Extramedullary hemangioblastoma of the spinal cord. Case report. J Neurosurg 48:461–464

    Article  CAS  PubMed  Google Scholar 

  73. Wizigmann-Voos S, Breier G, Risau W, Plate KH (1995) Up-regulation of vascular endothelial growth factor and its receptors in von Hippel-Lindau disease-associated and sporadic hemangioblastomas. Cancer Res 55:1358–1364

    CAS  PubMed  Google Scholar 

  74. Wizigmann-Voos S, Plate KH (1996) Pathology, genetics and cell biology of hemangioblastomas. Histol Histopathol 11:1049–1061

    CAS  PubMed  Google Scholar 

  75. Yoshii Y, Maki Y, Tomono Y, Nakamura T (1976) Cerebellar hemangioblastoma with multiple aneurysms. No To Shinkei 28:703–708 Japanese

    CAS  PubMed  Google Scholar 

  76. Yu JS, Short MP, Schumacher J, Chapman PH, Harsh GR 4th (1994) Intramedullary hemorrhage in spinal cord hemangioblastoma. Report of two cases. J Neurosurg 81:937–940

    Article  CAS  PubMed  Google Scholar 

  77. Zager EL, Shaver EG, Hurst RW, Flamm ES (2002) Distal anterior inferior cerebellar artery aneurysms. Report of four cases. J Neurosurg 97:692–696

    Article  PubMed  Google Scholar 

  78. Zhou L, Du G (2000) Diagnosis and surgical treatment of posterior fossa solid hemangioblastomas. Chin Med J (Engl) 113:129–132

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Javier Ros de San Pedro.

Additional information

Comments

Manolo Piccirilli, Rome, Italy

The authors report a very interesting case of massive hemorrhage in a 33-year-old male with histologically proven diagnosis of hemangioblastomas. A well-done literature review has been performed. This paper focuses the light on a very important neurosurgical issue, represented by spontaneous cerebral hemorrhages, and one of its possible etiologies, both intracranial and spinal.

Kazuhiro Hongo, Matsumoto, Japan

This is a paper reporting on the massive hemorrhage in hemangioblastoma with literature review. The authors are to be congratulated on their extensive and excellent literature review focusing on massive hemorrhage in hemangioblastoma.

As the hemangioblastoma is known to be a highly vascular tumor, for conducting resection, preoperative detailed evaluation of the feeding artery is essential, and at surgery careful maneuver is mandatory to manage the feeding artery at the early stage of surgery and to remove the lesion without entering the lesion itself.

As for the preoperative management, the authors give useful information: (1) subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances. (2) ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment.

Massive hemorrhage occurs rarely; however, we need to take care when managing a patient with a hemangioblastoma especially for a hypervascular one.

Takahito Miyazawa, Saitama, Japan

Cases of hemangioblastomas with bleeding are sometimes encountered. However, most neurosurgeons do not have time to think deeply about the scientific meaning of hemorrhage in hemangioblastomas. Authors discuss the issue of why, despite that there are pathological similarities between hemangioblastomas and AVMs, hemorrhage occurs less frequently in the former. Authors also discuss complications such as infarction, tumor swelling, and massive hemorrhage during or after preoperative embolization for hemangioblastomas. These issues are quite interesting and important for neurosurgeons. Since hemangioblastomas are not so frequent, the natural bleeding rate for them is not known. When neurosurgeons encounter a case of a hemangioblastoma without hemorrhage, they should know, as mentioned in the author’s conclusion, that a large size, a spinal–radicular location, and a solid type are factors associated with a higher bleeding rate.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Ros de San Pedro, J., Alarcón Rodríguez, F., Ferri Ñíguez, B. et al. Massive hemorrhage in hemangioblastomas. Neurosurg Rev 33, 11–26 (2010). https://doi.org/10.1007/s10143-009-0217-1

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10143-009-0217-1

Keywords

Navigation