Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease

Clinical article

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  • 1 Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland;
  • 2 Department of Neurological Surgery, George Washington University Medical Center, Washington, DC; and
  • 3 Department of Neurological Surgery, University of Virginia Health System, Charlottesville, Virginia
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Object

Brainstem hemangioblastomas are frequently encountered in patients with von Hippel-Lindau (VHL) disease. These tumors can cause significant morbidity, and their optimal management has not been defined. To better define the outcome and management of these tumors, the authors analyzed the long-term results in patients who underwent resection of brainstem hemangioblastomas.

Methods

Consecutive patients with VHL disease who underwent resection of brainstem hemangioblastomas with a follow-up of 12 months or more were included in this study. Serial functional assessments, radiographic examinations, and operative records were analyzed.

Results

Forty-four patients (17 male and 27 female) underwent 51 operations for resection of 71 brainstem hemangioblastomas. The most common presenting symptoms were headache, swallowing difficulties, singultus, gait difficulties, and sensory abnormalities. The mean follow-up was 5.9 ± 5.0 years (range 1.0–20.8 years). Immediately after 34 operations (66.7%), the patients remained at their preoperative functional status; they improved after 8 operations (15.7%) and worsened after 9 operations (17.6%) as measured by the McCormick scale. Eight (88.9%) of the 9 patients who were worse immediately after resection returned to their preoperative status within 6 months. Two patients experienced functional decline during long-term follow-up (beginning at 2.5 and 5 years postoperatively) caused by extensive VHL disease–associated CNS disease.

Conclusions

Generally, resection of symptomatic brainstem hemangioblastomas is a safe and effective management strategy in patients with VHL disease. Most patients maintain their preoperative functional status, although long-term decline in functional status may occur due to VHL disease–associated progression.

Abbreviations used in this paper: KPS = Karnofsky Performance Scale; NIH = National Institutes of Health; VHL = von Hippel-Lindau.

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Contributor Notes

Address correspondence to: Russell R. Lonser, M.D., Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Building 10, Room 3D20, Bethesda, Maryland 20892-1414. email: lonserr@ninds.nih.gov.

Please include this information when citing this paper: published online October 8, 2010; DOI: 10.3171/2010.9.JNS10839.

  • 1

    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 105:248255, 2006

    • Search Google Scholar
    • Export Citation
  • 2

    Asthagiri AR, , Mehta GU, , Zach L, , Li X, , Butman JA, & Camphausen KA, : Prospective evaluation of radiosurgery for hemangioblastomas in von Hippel-Lindau disease. Neuro Oncol 12:8086, 2010

    • Search Google Scholar
    • Export Citation
  • 3

    Baumgartner JE, & Wilson CB, Removal of posterior fossa and spinal hemangioblastomas. Wilson CB: Neurosurgical Procedures: Personal Approaches to Classic Operations Baltimore, Williams & Wilkins, 1992. 188199

    • Search Google Scholar
    • Export Citation
  • 4

    Filling-Katz MR, , Choyke PL, , Oldfield E, , Charnas L, , Patronas NJ, & Glenn GM, : Central nervous system involvement in Von Hippel-Lindau disease. Neurology 41:4146, 1991

    • Search Google Scholar
    • Export Citation
  • 5

    Fukushima T, , Sakamoto S, , Iwaasa M, , Hayashi S, , Yamamoto M, & Utsunomiya H, : Intramedullary hemangioblastoma of the medulla oblongata—two case reports and review of the literature. Neurol Med Chir (Tokyo) 38:489498, 1998

    • Search Google Scholar
    • Export Citation
  • 6

    Gläsker S, , Vortmeyer AO, , Lonser RR, , Lubensky IA, , Okamoto H, & Xia JB, : Proteomic analysis of hemangioblastoma cyst fluid. Cancer Biol Ther 5:549553, 2006

    • Search Google Scholar
    • Export Citation
  • 7

    Jawahar A, , Kondziolka D, , Garces YI, , Flickinger JC, , Pollock BE, & Lunsford LD: Stereotactic radiosurgery for hemangioblastomas of the brain. Acta Neurochir (Wien) 142:641645, 2000

    • Search Google Scholar
    • Export Citation
  • 8

    Kano H, , Niranjan A, , Mongia S, , Kondziolka D, , Flickinger JC, & Lunsford LD: The role of stereotactic radiosurgery for intracranial hemangioblastomas. Neurosurgery 63:443451, 2008

    • Search Google Scholar
    • Export Citation
  • 9

    Karnofsky DA, & Burchenal JH, The clinical evaluation of chemotherapeutic agents in cancer. MacLeod CM: Evaluation of Chemotherapeutic Agents New York, Columbia University Press, 1949. 199205

    • Search Google Scholar
    • Export Citation
  • 10

    Lamiell JM, , Salazar FG, & Hsia YE: von Hippel-Lindau disease affecting 43 members of a single kindred. Medicine (Baltimore) 68:129, 1989

    • Search Google Scholar
    • Export Citation
  • 11

    Latif F, , Tory K, , Gnarra J, , Yao M, , Duh FM, & Orcutt ML, : Identification of the von Hippel-Lindau disease tumor suppressor gene. Science 260:13171320, 1993

    • Search Google Scholar
    • Export Citation
  • 12

    Lewis EA: Gastroduodenal ulceration and haemorrhage of neurogenic origin. Br J Surg 60:279283, 1973

  • 13

    Lonser RR, , Glenn GM, , Walther M, , Chew EY, , Libutti SK, & Linehan WM, : von Hippel-Lindau disease. Lancet 361:20592067, 2003

  • 14

    Lonser RR, , Vortmeyer AO, , Butman JA, , Glasker S, , Finn MA, & Ammerman JM, : Edema is a precursor to central nervous system peritumoral cyst formation. Ann Neurol 58:392399, 2005

    • Search Google Scholar
    • Export Citation
  • 15

    Lundin P, & Pedersen F: Volume of pituitary macroadenomas: assessment by MRI. J Comput Assist Tomogr 16:519528, 1992

  • 16

    McCormick PC, , Torres R, , Post KD, & Stein BM: Intramedullary ependymoma of the spinal cord. J Neurosurg 72:523532, 1990

  • 17

    Moss JM, , Choi CY, , Adler JR Jr, , Soltys SG, , Gibbs IC, & Chang SD: Stereotactic radiosurgical treatment of cranial and spinal hemangioblastomas. Neurosurgery 65:7985, 2009

    • Search Google Scholar
    • Export Citation
  • 18

    Neumann HP, , Eggert HR, , Scheremet R, , Schumacher M, , Mohadjer M, & Wakhloo AK, : Central nervous system lesions in von Hippel-Lindau syndrome. J Neurol Neurosurg Psychiatry 55:898901, 1992

    • Search Google Scholar
    • Export Citation
  • 19

    Neumann HP, , Eggert HR, , Weigel K, , Friedburg H, , Wiestler OD, & Schollmeyer P: Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg 70:2430, 1989

    • Search Google Scholar
    • Export Citation
  • 20

    Park DM, , Zhuang Z, , Chen L, , Szerlip N, , Maric I, & Li J, : von Hippel-Lindau disease-associated hemangioblastomas are derived from embryologic multipotent cells. PLoS Med 4:e60, 2007

    • Search Google Scholar
    • Export Citation
  • 21

    Parker F, , Aghakhani N, , Ducati LG, , Yacubian-Fernandes A, , Silva MV, & David P, : Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups. J Neurooncol 93:133137, 2009

    • Search Google Scholar
    • Export Citation
  • 22

    Patrice SJ, , Sneed PK, , Flickinger JC, , Shrieve DC, , Pollock BE, & Alexander E III, : Radiosurgery for hemangioblastoma: results of a multiinstitutional experience. Int J Radiat Oncol Biol Phys 35:493499, 1996

    • Search Google Scholar
    • Export Citation
  • 23

    Rachinger J, , Buslei R, , Prell J, & Strauss C: Solid haemangioblastomas of the CNS: a review of 17 consecutive cases. Neurosurg Rev 32:3748, 2009

    • Search Google Scholar
    • Export Citation
  • 24

    Song DK, & Lonser RR: Pathological satiety caused by brainstem hemangioblastoma. Case report. J Neurosurg Pediatr 2:397401, 2008

  • 25

    Tago M, , Terahara A, , Shin M, , Maruyama K, , Kurita H, & Nakagawa K, : Gamma knife surgery for hemangioblastomas. J Neurosurg 102:Suppl 171174, 2005

    • Search Google Scholar
    • Export Citation
  • 26

    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 98:8294, 2003

    • Search Google Scholar
    • Export Citation
  • 27

    Wang C, , Zhang J, , Liu A, & Sun B: Surgical management of medullary hemangioblastoma. Report of 47 cases. Surg Neurol 56:218227, 2001

  • 28

    Wang EM, , Pan L, , Wang BJ, , Zhang N, , Zhou LF, & Dong YF, : The long-term results of gamma knife radiosurgery for hemangioblastomas of the brain. J Neurosurg 102:Suppl 225229, 2005

    • Search Google Scholar
    • Export Citation
  • 29

    Weil RJ, , Lonser RR, , DeVroom HL, , Wanebo JE, & Oldfield EH: Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg 98:95105, 2003

    • Search Google Scholar
    • Export Citation
  • 30

    Zhou LF, , Du G, , Mao Y, & Zhang R: Diagnosis and surgical treatment of brainstem hemangioblastomas. Surg Neurol 63:307316, 2005

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