Surgical management of brainstem hemangioblastomas in patients with von Hippel—Lindau disease

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Object. Hemangioblastomas of the brainstem constitute 5 to 10% of central nervous system (CNS) tumors in patients with von Hippel—Lindau (VHL) disease. At present, optimal management of brainstem hemangioblastomas associated with VHL disease is incompletely defined. In an attempt to clarify some of the uncertainty about the operative treatment of these lesions and its outcome, the authors reviewed all cases of VHL disease in which resection of brainstem hemangioblastomas was performed at the National Institutes of Health during a 10-year period.

Methods. Twelve consecutive patients with VHL disease (six male and six female patients [mean age 31.7 ± 9 years; range 15–46 years]) who underwent 13 operations to remove 17 brainstem hemangioblastomas were included in this study (mean follow-up period, 88.4 ± 37.4 months; range 37–144 months). Serial examinations, hospital charts, magnetic resonance images, and operative records were reviewed. To evaluate clinical course, clinical grades were assigned to each patient before and after surgery.

Preoperative neurological function was the best predictor of long-term outcome. In addition, patients who underwent CNS surgeries for hemangioblastomas were more likely to improve or to remain neurologically stable. Tumor or cyst size, the presence of a cyst, or the location of the tumor (intramedullary, extramedullary, or mixed; posterior medullary, obex, or lateral) did not affect outcome. No patient was neurologically worse after brainstem surgery. At long-term follow-up review (mean 88.4 months), only one patient had declined neurologically and this was due to the cumulative neurological effects caused by eight additional hemangioblastomas of the spinal cord and their surgical treatment.

Conclusions. Brainstem hemangioblastomas in patients with VHL disease can be removed safely; they generally should be resected when they become symptomatic or when the tumor has reached a size such that further growth will increase the risks associated with surgery, or in the presence of an enlarging cyst. Magnetic resonance imaging is usually sufficient for preoperative evaluation and presurgical embolization is unnecessary. The goal of surgery is complete resection of the lesion before the patient experiences a disabling neurological deficit.

Article Information

Address reprint requests to: Robert J. Weil, M.D., Surgical Neurology Branch, NINDS, NIH, Building 10, Room 5D 37, Bethesda, Maryland 20892–1414. email: weilr@ninds.nih.gov.

© AANS, except where prohibited by US copyright law.

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    Case 12. A and B: Contrast-enhanced sagittal (A) and axial (B) MR images of the brain obtained 6 months before surgery, revealing a small hemangioblastoma and no cyst. C and D: Sagittal (C) and axial (D) MR images obtained immediately before surgery, demonstrating enlargement of the tumor and rapid development of a cyst with brainstem compression. E and F: Intraoperative photographs showing a bulging medulla oblongata, without evident tumor (E); an enlarged draining vein is present in the midline (superior aspect of the medulla at top of panel). After incising the pia mater and gently opening the midline raphe, the tumor capsule is exposed, which permits complete resection by dissection of the interface between the tumor capsule and surrounding neural tissue (F). G: Postoperative axial MR image confirming complete resection of the tumor and disappearance of the brainstem cyst 2 weeks after surgery.

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    Case 2. A–C: Axial (A), sagittal (B), and coronal (C) contrast-enhanced MR images of the brain obtained preoperatively. D and E: Intraoperative photographs showing a principally extramedullary tumor (superior at top of panel). The tonsils have been elevated to expose the tumor (D). Subpial dissection of the inferior margin of the tumor after incision of the pia mater at its junction with the tumor margin (E). Removing the large extramedullary tumor revealed a second, smaller tumor that was also removed. F and G: Postoperative axial (F) and sagittal (G) MR images obtained 6 (F) and 12 (G) months after surgery, demonstrating complete tumor removal and disappearance of the cyst.

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    Case 6. A and B: Coronal MR images obtained before (A) and after (B) addition of contrast material, revealing a large hemangioblastoma and cyst. C and D: Preoperative sagittal MR views of the tumor obtained before (C) and after (D) addition of contrast material, demonstrating a large tumor along the posterior margin of the cyst and a small focus of enhancement (arrow) in D along the most caudal pole of the cyst at the midpoint of the odontoid. E–G: Contrast-enhanced MR images obtained 6 months after the first surgery, in which the large hemangioblastoma was removed, revealing a persistent cyst. H: Intraoperative photograph obtained at repeated surgery, showing a small orange-red mural nodule within the operative mirror (arrowhead; superior is at the top of the panel and the patient's left side is situated to the left of the panel). The mirror reflects the small tumor at the most inferior extent of the intramedullary cyst. I: Intraoperative photograph of the 2-mm-diameter tumor before resection and after entry into the cyst. J and K: Contrast-enhanced MR images obtained 1 month after surgery, confirming removal of the tumor and decompression of the cyst.

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