Top

Published in:

01-01-2009 | Original Article

Solid haemangioblastomas of the CNS: a review of 17 consecutive cases

Authors: Jens Rachinger, Rolf Buslei, Julian Prell, Christian Strauss

Published in: Neurosurgical Review | Issue 1/2009

Abstract

A retrospective study on solid central nervous system haemangioblastomas was performed to characterize clinical features, treatment strategies and outcome in these rare lesions. Between 1993 and 2006 23, solid haemangioblastomas were surgically removed in 17 patients. Eight lesions were located within pons Varolii and medulla oblongata, six within the cerebellar hemispheres and three in the cerebellopontine angle. Three haemangioblastomas were located supratentorially and three within the spinal cord. All patients except two underwent pre-operative magnetic resonance imaging (MRI). Post-operative digital subtraction angiography and/or MRI was performed in all surviving patients. Except for spinal cord lesions, rather unsystematic clinical symptoms were observed. Twenty-two tumours could be resected completely. Two patients with brainstem lesions died within 10 weeks after surgery from infectious complications. Persistent new neurological deficits occurred in two patients. Three patients underwent radiosurgery prior to or following the surgical procedure. Solid haemangioblastomas represent a surgical challenge due to their arteriovenous malformation-like vascularisation and their frequent location in eloquent areas. Surgery is the therapy of choice. Circumferential dissection with devascularization and en bloc removal yields good functional results. A location within the brainstem carries the most unfavourable prognosis.

Biondi A, Ricciardi GK, Faillot T, Capelle L, Van Effenterre R, Chiras J (2005) Hemangioblastomas of the lower spinal region: report of four cases with preoperative embolization and review of the literature. Am J Neuroradiol 26:936–945PubMed

Chang SD, Meisel JA, Hancock SL, Martin DP, Mc Manus M, Adler JR Jr (1998) Treatment of hemangioblastomas in von Hippel–Lindau disease with linear-accelerator-based radiosurgery. Neurosurgery 43:28–35PubMedCrossRef

Chen F, Kishida T, Yao M, Hustad T, Glavac D, Dean M, Gnarra JR (1995) Germline mutations in the von Hippel–Lindau disease tumor suppressor gene: correlations with phenotype. Hum Mutat 5:66–75PubMedCrossRef

Chen F, Slife, Kishida T, Mulvihill J, Tishermann SE, Zbar B (1996) Genotype-phenotype correlation in von Hippel–Lindau disease: identification of a mutation associated with VHL type 2A. J Med Genet 33:716–717PubMedCrossRef

Chou SN, Erickson DL, Ortiz-Suarez JH (1975) Surgical treatment of vascular lesions in the brain stem. J Neurosurg 42:23–31PubMed

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(1):55–62PubMedCrossRef

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 106:994–998PubMedCrossRef

Dow GR, Sim DW, O’Sullivan MG (2002) Excision of large solid haemangioblastomas of the cerebellopontine angle by a skull base approach. Br J Neurosurg 16(2):168–171PubMedCrossRef

Eskridge JM, McAuliffe W, Harris B, Kim DK, Scott J, Winn HR (1996) Preoperative endovascular embolization of craniospinal hemangioblastomas. Am J Neuroradiol 17(3):525–531PubMed

10.

Filling-Katz MR, Choyke PL, Oldfield E, Charnas L, Patronas NJ, Glenn GM, Gorin MB, Morgan JK, Linehan WM, Seizinger BR (1991) Central nervous system involvement in von Hippel–Lindau disease. Neurology 41:41–46PubMed

11.

Glenn GM, Linehan WM, Hosoe S, Latif F, Yao M, Choyke P, Gorin MB et al (1992) Screening for von Hippel–Lindau disease by DNA polymorphism analysis. JAMA 267:1226–1231PubMedCrossRef

12.

Jawahar A, Kondziolka D, Garces YI, Flickinger JC, Pollock BE, Lunsford LD (2000) Stereotactic radiosurgery for hemangioblastomas of the brain. Acta Neurochir (Wien) 142(6):641–644CrossRef

13.

Julow J, Balint K, Gortvai P, Pasztor E (1994) Posterior fossa haemangioblastomas. Acta Neurochir (Wien) 128(1–4):109–114CrossRef

14.

Ho YS, Plets C, Goffin J, Dom R (1990) Hemangioblastoma of the lateral ventricle. Surg Neurol 33:407–412PubMedCrossRef

15.

Hosoe S, Brauch H, Latif F, Glenn G, Daniel F, Bale S, Choyke P et al (1990) Localization of the von Hippel–Lindau disease gene to a small region of chromosome 3. Genomics 8:634–640PubMedCrossRef

16.

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 67:137–143PubMedCrossRef

17.

Latif F, Tory K, Gnarra J, Yao M, Duh FM, Orcutt ML, Stackhouse T et al (1993) Identification of the von Hippel–Lindau disease tumor suppressor gene. Science 260:1317–1320PubMedCrossRef

18.

Lonser R, Weil R, Wanebo J, DeVroom H, Oldfield E (2003) Surgical management of spinal cord hemangioblastomas in patients with von Hippel–Lindau disease. J Neurosurg 98:106–116PubMed

19.

Machein MR, Plate KH (2000) VEGF in brain tumors. J Neurooncol 50(1–2):109–120PubMedCrossRef

20.

Mans DA, Lolkema MP, van Beest M, Daenen LG, Voest EE, Giles RH (2008) Mobility of the von Hippel–Lindau tumour suppressor protein is regulated by kinesin-2. Exp Cell Res 14:1229–1236CrossRef

21.

Naik RTS, Purohit AK, Dinakar I, Ratnakar KS (1995) Hemangioblastoma of the IV. ventricle. Childs Nerv Syst 11:499–500PubMedCrossRef

22.

Neumann HP, Wiestler OD (1994) Von Hippel–Lindau disease: a syndrome providing insights into growth control and tumorigenesis. Nephrol Dial Transplant 9:1832–1833PubMed

23.

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

24.

Okawara SH (1973) Solid cerebellar hemangioblastoma. J Neurosurg 39:514–518PubMed

25.

Palmer JJ (1972) Hemangioblastomas. A review of 81 cases. Acta Neurochir 27:125–148CrossRef

26.

Pan L, Wang EM, Wang BJ, Zhou LF, Zhan N, Cai PW, Da JZ (1998) Gamma knife radiosurgery for hemangioblastomas. Stereotact Funct Neurosurg 70(Suppl):179–186PubMedCrossRef

27.

Rawe SE, Van Gilder JC, Rothman SLG (1978) Radiographic diagnostic evaluation and surgical treatment of multiple cerebellar, brain stem, and spinal cord hemangioblastomas. Surg Neurol 9:337–341PubMed

28.

Resche F, Moisan JP, Mantoura J, De Kersaint-Gilly A, Andre MJ, Perrin-Resche I, Menegalli-Bogelli D, Lajat Y, Richard S (1993) Haemangioblastoma, haemangioblastomatosis, and von Hippel–Lindau disease. Adv Technol Stand Neurosurg 20:197–304

29.

Rodesch G, Gaillard S, Loiseau H, Brotchi J (2008) Embolization of intradural vascular spinal cord tumors: Report of five cases and review of the literature. Neuroradiology 50:145–151PubMedCrossRef

30.

Russel DS, Rubinstein WLJ (eds) (1989) Pathology of tumors of the nervous system. Arnold, 5th edn, London

31.

Ryu SI, Kim DH, Chang SD (2003) Stereotactic radiosurgery for hemangiomas and ependymomas of the spinal cord. Neurosurg Focus 15(5):E10PubMedCrossRef

32.

Sanford RA, Smith RA (1986) Haemangioblastomas of the cervico-medullary junction. J Neurosurg 64:317–321PubMed

33.

Santarpia L, Sarlis NJ, Santarpia M, Sherman SI, Trimarchi F, Benvenga S (2007) Mosaicism in von Hippel–Lindau disease: an event important to recognize. J Cell Mol Med 11:1408–1415PubMed

34.

Seizinger BR, Rouleau GA, Ozelius LJ, Lane AH, Farmer GE, Lamiell JM, Haines J et al (1988) Von Hippel–Lindau disease maps to the region of chromosome 3 associated with renal cell carcinoma. Nature 332:268–269PubMedCrossRef

35.

Singounas EG (1978) Hemangioblastomas of the central nervous system. Acta Neurochir (Wien) 44:107–113CrossRef

36.

Spetzger U, Bertalanffy H, Huffmann B, Mayfrank L, Reul J, Gilsbach JM (1996) Hemangioblastomas of the spinal cord and the brainstem: diagnostic and therapeutic features. Neurosurg Rev 19:147–151PubMedCrossRef

37.

Symon L, Murota T, Pell M, Bordi L (1993) Surgical management of haemangioblastoma of the posterior fossa. Acta Neurochir (Wien) 120(3–4):103–110CrossRef

38.

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

39.

Takeuchi S, Tanaka R, Fujii Y, Abe H, Ito Y (2001) Surgical treatment of hemangioblastomas with presurgical endovascular embolization. Neurol Med Chir (Tokyo) 41(5):246–251CrossRef

40.

Tampieri D, Leblanc R, TerBrugge K (1993) Preoperative embolization of brain and spinal hemangioblastomas. Neurosurgery 33(3):502–505PubMedCrossRef

41.

Van Velthoven V, Reinacher PC, Klisch J, Neumann HP, Glasker S (2003) Treatment of intramedullary hemangioblastomas, with special attention to von Hippel–Lindau disease. Neurosurgery 53(6):1306–1313PubMedCrossRef

42.

Vazquez-Anon V, Botella C, Beltran A, Solera M, Piquer J (1997) Preoperative embolization of solid cervicomedullary junction hemangioblastomas: report of two cases. Neuroradiology 39(2):86–89PubMedCrossRef

43.

Wanebo JE, Lonser R, Glenn G, Oldfield E (2003) The natural history of hemangioblastomas of the central nervous system in patients with von Hippel–Lindau disease. J Neurosurg 98:82–94PubMed

44.

Wang C, Zhang J, Liu A, Sun B (2001) Surgical management of medullary hemangioblastoma. Report of 47 cases. Surg Neurol 56(4):218–226PubMedCrossRef

45.

Wang EM, Pan L, Wang BJ, Zhang N, Zhou LF, Dong YF, Dai JZ, Cai PW, Chen H (2005) The long-term results of gamma knife radiosurgery for hemangioblastomas of the brain. J Neurosurg 102(Suppl):225–229PubMed

46.

Weil RJ, Lonser RR, DeVroom HL, Wanebo JE, Oldfield EH (2003) Surgical management of brainstem hemangioblastomas in patients with von Hippel–Lindau disease. J Neurosurg 98(1):95–105PubMed

47.

Yasargil MG, Antic J, Laciga R, De Preux J, Fideler RW, Boone SC (1976) The microsurgical removal of intramedullary spinal hemangioblastomas: Report of twelve cases and a review of the literature. Surg Neurol 6:141–146

48.

Young S, Richardson AE (1987) Solid hemangioblastomas of the posterior fossa: radiological features and results of surgery. J Neurol Neurosurg Psychiatry 50:155–158PubMedCrossRef

49.

Zbar B, Kishida T, Chen F, Schmidt L, Maher ER, Richards FM, Crossey PA et al (1996) Germline mutations in the Von Hippel–Lindau disease (VHL) gene in families from North America, Europe, and Japan. Hum Mutat 8:348–357PubMedCrossRef

50.

Zimmermann M, Seifert V, Schreyer V, Stolke D, Dietz H (1997) Hemangioblastoma: Description of a disease picture and report of 41 cases. Zentralbl Neurochir 58(1):1–6PubMed

51.

Zhou LF, Du G, Mao Y, Zhang R (2005) Diagnosis and surgical treatment of brainstem hemangioblastomas. Surg Neurol 63(4):307–315PubMedCrossRef

Title: Solid haemangioblastomas of the CNS: a review of 17 consecutive cases
Authors: Jens Rachinger
Rolf Buslei
Julian Prell
Christian Strauss
Publication date: 01-01-2009
Publisher: Springer-Verlag
Published in: Neurosurgical Review / Issue 1/2009
Print ISSN: 0344-5607
Electronic ISSN: 1437-2320
DOI: https://doi.org/10.1007/s10143-008-0166-0

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Solid haemangioblastomas of the CNS: a review of 17 consecutive cases

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 1/2009

Surgical outcomes of lateral approach for jugular foramen schwannoma: postoperative facial nerve and lower cranial nerve functions

Surgical management of foramen magnum meningiomas

Spinal radiosurgery: technology and clinical outcomes

Management of skin erosion following deep brain stimulation

Current strategies for the treatment of intracranial atherosclerotic internal carotid artery stenosis

Cerebrovascular insufficiency as the criterion for revascularization procedures in selected patients: a correlation study of xenon contrast-enhanced CT and PWI