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

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Object. Von Hippel—Lindau (VHL) disease is an autosomal-dominant disorder frequently associated with hemangioblastomas of the spinal cord. Because of the slow progression, protean nature, and high frequency of multiple spinal hemangioblastomas associated with VHL disease, the surgical management of these lesions is complex. Because prior reports have not identified the factors that predict which patients with spinal cord hemangioblastomas need surgery or what outcomes of this procedure should be expected, the authors have reviewed a series of patients with VHL disease who underwent resection of spinal hemangioblastomas at a single institution to identify features that might guide surgical management of these patients.

Methods. Forty-four consecutive patients with VHL disease (26 men and 18 women) who underwent 55 operations with resection of 86 spinal cord hemangioblastomas (mean age at surgery 34 years; range 20–58 years) at the National Institutes of Health were included in this study (mean clinical follow up 44 months). Patient examination, review of hospital charts, operative findings, and magnetic resonance imaging studies were used to analyze surgical management and its outcome. To evaluate the clinical course, clinical grades were assigned to patients before and after surgery. Preoperative neurological status, tumor size, and tumor location were predictive of postoperative outcome. Patients with no or minimal preoperative neurological dysfunction, with lesions smaller than 500 mm3, and with dorsal lesions were more likely to have no or minimal neurological impairment. Syrinx resolution was the result of tumor removal and was not influenced by whether the syrinx cavity was entered.

Conclusions. Spinal cord hemangioblastomas can be safely removed in the majority of patients with VHL disease. Generally in these patients, hemangioblastomas of the spinal cord should be removed when they produce symptoms or signs.

Article Information

Address reprint requests to: Russell R. Lonser, M.D., Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, Building 10, Room 5D37, Bethesda, Maryland 20892–1414. email: lonserr@ninds.nih.gov.

© AANS, except where prohibited by US copyright law.

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Figures

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    Artist's depiction of the steps of microsurgical resection of spinal cord hemangioblastomas. This drawing illustrates the intradural portion of the operation for resection of these lesions. Using an operating microscope, the associated vessels and tumor are identified. The tumor is exposed by reflecting the overlying vessels with gentle traction (a). The junction between the pia mater and the tumor capsule is identified and incised using a diamond knife and microscissors (b). Dissection of the tumor is continued deeper and circumferentially at the tumor capsule—spinal cord interface by using bipolar microforceps (c). After circumferential dissection of the tumor margin is complete, the pole inferior to the hemangioblastoma is reflected by applying gentle suction on a cottonoid to expose the underlying vessels (d).

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    Schematic drawing showing hemangioblastoma distribution. Dots represent the regional distribution of the 86 spinal cord hemangioblastomas that were resected in this study.

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    Charts correlating neurological status before surgery and at final outcome (after surgery at last follow-up examination). Eighty-four percent of patients remained at the same grade, 7% improved (one grade), and 9% worsened (one or more grades) as of the final clinical assessment. Open circles indicate the four patients who underwent resection of ventrally located spinal hemangioblastomas. Numbers represent McCormick grades.

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    A: Relationship between tumor size and final outcome. This chart shows the postoperative clinical outcome of each patient (dots) distributed by tumor size (the largest tumor in each patient was used for the analysis). Patients harboring tumors with volumes smaller than 500 mm3 (diameter < 10 mm assuming a spherical tumor) did not worsen neurologically after surgery. B: Relationship between tumor size and presence of syrinx. This chart shows the wide variation in tumor sizes (taken as the maximum lesion size) that were (range 13–13,800 mm3) and were not (range 32–500 mm3) associated with a syrinx. C: Relationship between clinical outcome (McCormick grade) and the presence of a syrinx. This chart shows the preoperative (black dots) and postoperative (open boxes) clinical grades of patients with and without syringes. Before surgery, a patient without a syrinx generally functioned at a better clinical grade (83% of patients with Grade I and 17% with Grade II) than a patient with a syrinx (45% of patients with Grade I, 37% with Grade II, 13% with Grade III, and 5% with Grade IV). Overall, patients tended to maintain a stable clinical grade as of their last follow-up examination.

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    Case A. Contrast-enhanced sagittal MR images of the cervical spine demonstrating the progressive nature and multiplicity of spinal hemangioblastomas in a patient with VHL disease. A: Eighteen months before admission to the NIH, imaging reveals a syrinx associated with hemangioblastomas located at C-1 and C5–6. B: Image obtained 14 months later, when the patient was having increasingly severe headaches, demonstrating enlargement of the cervical tumors. The patient underwent resection of the C-1 tumor because it was thought to be the underlying cause of her syringomyelia. C: Image obtained 6 months after resection of the C-1 hemangioblastoma. There is progressive enlargement of the C5–6 lesion and no change in the cervical syrinx. The patient underwent resection of this lesion. D: Follow-up image obtained 8 months after removal of the C5–6 hemangioblastoma confirming resolution of the syrinx cavity. Clinically, this patient has remained at Grade I throughout the follow-up period (13.6 months).

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    Case B. Magnetic resonance images of the cervical spine demonstrating the rapid resolution of spinal cord edema after resection of associated hemangioblastomas. A: Preoperative sagittal T2-weighted image revealing spinal cord edema. B: Preoperative sagittal contrast-enhanced image demonstrating cord swelling and an associated lesion between C-5 and C-6. C: Preoperative axial contrast-enhanced images depicting edema at C-6. D: Sagittal unenhanced T2-weighted image obtained on postoperative Day 7 demonstrating resolution of the edema. E: Sagittal contrast-enhanced image depicting resection of the hemangioblastomas and resolution of edema throughout the follow-up studies (8 months). F: Axial unenhanced image obtained on postoperative Day 7 confirming complete resolution of the edema.

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