Sacral hemangioblastoma in a patient with von Hippel-Lindau disease. Case report and review of the literature.

Ryszard M. Pluta, Scott D. Wait, John A. Butman, Kathleen A. Leppig, Alexander Vortmeyer, Edward H. Oldfield, Russell R. Lonser

Research output: Contribution to journalReview article

12 Citations (Scopus)

Abstract

Hemangioblastomas are histologically benign neoplasms that occur sporadically or as part of von Hippel-Lindau disease. Hemangioblastomas may occur anywhere along the neuraxis, but sacral hemangioblastomas are extremely rare. To identify features that will help guide the operative and clinical management of these lesions, the authors describe the management of a large von Hippel-Lindau disease-associated sacral hemangioblastoma and review the literature. The authors present the case of a 38-year-old woman with von Hippel-Lindau disease and a 10-year history of progressive back pain, as well as left lower-extremity pain and numbness. Neurological examination revealed decreased sensation in the left S-1 and S-2 dermatomes. Magnetic resonance imaging demonstrated a large enhancing lesion in the sacral region, with associated erosion of the sacrum. The patient underwent arteriography and embolization of the tumor and then resection. The histopathological diagnosis was consistent with hemangioblastoma and showed intrafascicular tumor infiltration of the S-2 nerve root. At 1-year follow-up examination, pain had resolved and numbness improved. Sacral nerve root hemangioblastomas may be safely removed in most patients, resulting in stabilization or improvement in symptomatology. Generally, hemangioblastomas of the sacral nerve roots should be removed when they cause symptoms. Because they originate from the nerve root, the nerve root from which the hemangioblastoma originates must be sacrificed to achieve complete resection.

Original languageEnglish (US)
JournalNeurosurgical focus
Volume15
Issue number2
StatePublished - Jan 1 2003
Externally publishedYes

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Hemangioblastoma
von Hippel-Lindau Disease
Hypesthesia
Sacrococcygeal Region
Pain
Sacrum
Neoplasms
Neurologic Examination
Back Pain
Lower Extremity
Angiography
Magnetic Resonance Imaging

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

Cite this

Pluta, R. M., Wait, S. D., Butman, J. A., Leppig, K. A., Vortmeyer, A., Oldfield, E. H., & Lonser, R. R. (2003). Sacral hemangioblastoma in a patient with von Hippel-Lindau disease. Case report and review of the literature. Neurosurgical focus, 15(2).

Sacral hemangioblastoma in a patient with von Hippel-Lindau disease. Case report and review of the literature. / Pluta, Ryszard M.; Wait, Scott D.; Butman, John A.; Leppig, Kathleen A.; Vortmeyer, Alexander; Oldfield, Edward H.; Lonser, Russell R.

In: Neurosurgical focus, Vol. 15, No. 2, 01.01.2003.

Research output: Contribution to journalReview article

Pluta, RM, Wait, SD, Butman, JA, Leppig, KA, Vortmeyer, A, Oldfield, EH & Lonser, RR 2003, 'Sacral hemangioblastoma in a patient with von Hippel-Lindau disease. Case report and review of the literature.', Neurosurgical focus, vol. 15, no. 2.
Pluta, Ryszard M. ; Wait, Scott D. ; Butman, John A. ; Leppig, Kathleen A. ; Vortmeyer, Alexander ; Oldfield, Edward H. ; Lonser, Russell R. / Sacral hemangioblastoma in a patient with von Hippel-Lindau disease. Case report and review of the literature. In: Neurosurgical focus. 2003 ; Vol. 15, No. 2.
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