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

2003 ◽  
Vol 98 (1) ◽  
pp. 95-105 ◽  
Author(s):  
Robert J. Weil ◽  
Russell R. Lonser ◽  
Hetty L. Devroom ◽  
John E. Wanebo ◽  
Edward H. Oldfield
Keyword(s):  
Magnetic Resonance ◽  
Preoperative Evaluation ◽  
Magnetic Resonance Images ◽  
Long Term Outcome ◽  
Content Type ◽  
Von Hippel Lindau ◽  
Vhl Disease ◽  
Fine Print

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.

Long-term hearing preservation after surgery for vestibular schwannoma

2005 ◽  
Vol 102 (1) ◽  
pp. 6-9 ◽  
Author(s):  
Simone A. Betchen ◽  
Jane Walsh ◽  
Kalmon D. Post
Keyword(s):  
Hearing Preservation ◽  
Magnetic Resonance Images ◽  
Class I ◽  
Repeated Testing ◽  
Content Type ◽  
Factors Influencing ◽  
Long Term Preservation ◽  
Fine Print

Object. Vestibular schwannomas (VSs) are now amenable to resection with excellent hearing preservation rates. It remains unclear whether immediately postoperative hearing is a durable result and will not diminish over time. The aim of this study was to determine the rate of long-term preservation of functional hearing following surgery for a VS and to examine factors influencing hearing preservation. Methods. All patients eligible for hearing preservation (Gardner—Robertson Class I or II) who had undergone resection of a VS by a single surgeon were reviewed retrospectively. Follow-up audiograms and magnetic resonance images were obtained. Of 142 patients deemed eligible for hearing preservation surgery, 38 had immediate postoperative hearing confirmed by an audiogram. In these patients with preserved hearing, the audiographic results demonstrated functional hearing in 30 (85.7%) of 35 patients who underwent repeated testing at a mean follow-up time of 7 years. Delayed hearing loss occurred in five (14.3%) of the 35 patients and did not correlate significantly with the size of the tumor. Hearing improved one Gardner—Robertson class postoperatively in three (7.9%) of the 38 patients. Conclusions. Long-term functional hearing was maintained in 85.7% of patients when it was preserved immediately postoperatively and the result was independent of tumor size. The results of this study emphasize that long-term preservation of functional hearing is a realistic goal following VS surgery and should be attempted in all patients in whom preoperative hearing is determined to be Gardner—Robertson Class I or II.

Surgical treatment for cervical spondylitic myelopathy

1995 ◽  
Vol 82 (5) ◽  
pp. 745-751 ◽  
Author(s):  
Michael J. Ebersold ◽  
Michel C. Pare ◽  
Lynn M. Quast
Keyword(s):  
Cord Compression ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Content Type ◽  
Posterior Laminectomy ◽  
Anterior Decompression And Fusion ◽  
Anterior Group ◽  
Fine Print

✓ The long-term outcome of cervical spondylitic myelopathy after surgical treatment was retrospectively reviewed and critically evaluated in 100 patients with documented cervical myelopathy treated between 1978 and 1988 at our institution. Eighty-four patients were available for long-term study. The median duration of follow up was 7.35 years (range 3 to 9.5 years). There were 67 men and 17 women; their ages ranged from 27 to 86 years. The duration of preoperative symptoms ranged from 1 month to 10 years. Preoperative functional grade as evaluated with the Nurick Scale for the group was 2.1. Thirty-three patients with primarily anterior cord compression, one- or two-level disease, or a kyphotic neck deformity were treated by anterior decompression and fusion. Fifty-one patients with primarily posterior cord compression and multiple-level disease were treated by posterior laminectomy. There was no difference in the preoperative functional grade in these two groups. The patients in the posterior treatment group were older (59 vs 55 years). There was no surgical mortality from the operative procedures; morbidity was 3.6%. Of the 33 patients undergoing anterior decompression and fusion, 24 showed immediate functional improvement and nine were unchanged. Of the 51 patients who underwent posterior laminectomy, 35 demonstrated improvement, 11 were unchanged, and five were worse. Six patients, one in the anterior group and five in the posterior group, demonstrated early deterioration. Late deterioration occurred from 2 to 68 months postoperatively. Four (12%) patients who had undergone anterior procedures had additional posterior procedures, and seven (13.7%) patients who had undergone posterior procedures had additional decompressive surgery. The final functional status at last follow-up examination for the 33 patients in the anterior group was improved in 18, unchanged in nine, and deteriorated in six. Of the 51 patients who underwent posterior decompression, 19 benefited from the surgery, 13 were unchanged, and 19 were worse at last follow up than before their initial surgical procedure. Age, severity of disease, number of levels operated, and preoperative grade were not predictive of outcome. The only factor related to potential deterioration was the duration of symptoms preoperatively. The results indicate that with anterior or posterior decompression, long-term outcome is variable, and a subgroup of patients, even after adequate decompression and initial improvement, will have late functional deterioration.

Percutaneous endoscopic treatment of suprasellar arachnoid cysts: ventriculocystostomy or ventriculocystocisternostomy?

1996 ◽  
Vol 84 (4) ◽  
pp. 696-701 ◽  
Author(s):  
Philippe Decq ◽  
Pierre Brugières ◽  
Caroline Le Guerinel ◽  
Michel Djindjian ◽  
Yves Kéravel ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Flow Dynamics ◽  
The Other ◽  
Arachnoid Cysts ◽  
Csf Flow ◽  
Content Type ◽  
Secondary Closure ◽  
Fine Print

✓ The use of an endoscope in the treatment of suprasellar arachnoid cysts provides an opening of the upper and lower cyst walls, thereby allowing the surgeon to perform a ventriculocystostomy (VC) or a ventriculocystocysternostomy (VCC). To discover which procedure is appropriate, magnetic resonance (MR)—imaged cerebrospinal fluid (CSF) flow dynamics in two patients were analyzed, one having undergone a VC and the other a VCC using a rigid endoscope. Magnetic resonance imaging studies were performed before and after treatment, with long-term follow-up periods (18 months and 2 years). The two patients were reoperated on during the follow-up period because of slight headache recurrence in one case and MR—imaged CSF flow dynamics modifications in the other. In each case surgery confirmed the CSF flow dynamics modifications appearing on MR imaging. In both cases, long-term MR imaging follow-up studies showed a secondary closing of the upper wall orifice. After VCC, however, the lower communication between the cyst and the cisterns remained functional. The secondary closure of the upper orifice may be explained as follows: when opened, the upper wall becomes unnecessary and tends to return to a normal shape, leading to a secondary closure. The patent sylvian aqueduct aids this phenomenon, as observed after ventriculostomy when the aqueduct is secondarily functional. The simplicity of the VCC performed using endoscopic control, which is the only procedure to allow the opening in the cyst's lower wall to remain patent, leads the authors to advocate this technique in the treatment of suprasellar arachnoid cysts.

The long-term results of gamma knife radiosurgery for hemangioblastomas of the brain

2005 ◽  
Vol 102 ◽  
pp. 225-229 ◽  
Author(s):  
En-Min Wang ◽  
Li Pan ◽  
Bing-Jiang Wang ◽  
Nan Zhang ◽  
Liang-Fu Zhou ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Tumor Control ◽  
Content Type ◽  
Prescription Dose ◽  
The Mean ◽  
Vhl Disease ◽  
The Brain ◽  
Fine Print

Object. The authors assessed the long-term result of gamma knife surgery (GKS) for hemangioblastomas of the brain (HABs) and show histopathological findings after GKS. Methods. Thirty-five patients, 28 men and seven women, with a mean age of 36 years underwent GKS. Eighteen patients presented with multiple tumors and 17 with a solitary tumor. Twenty-one patients had von Hippel—Lindau (VHL) disease. The mean tumor diameter was 13 mm (range 5–55 mm). The mean follow up after GKS was 66 months (range 24–114 months). The mean prescription dose was 17.2 Gy (range 12–24 Gy) at the tumor margin. For tumors close to or within the brainstem a prescription dose of 12 to 13 Gy was used. At the most recent follow up, 29 patients were alive, six were dead, and satisfactory tumor control had been achieved in 29. A stable or improved neurological status was obtained in 21 patients. Eight patients underwent open surgery because of tumor-associated cyst enlargement or the development of new tumors after GKS. Seven patients developed new tumors and five of them required a second GKS. The 1-year tumor control rate was 94%; 2 years, 85%; 3 years, 82%; 4 years, 79%; and 5 years, 71%. Histopathology showed that no tumor cells were found and there was degeneration and necrosis in a tumor nodule 48 months after GKS with a prescription dose of 18 Gy. Conclusions. Gamma knife surgery was a useful choice for small- or medium-sized, solid HAB in the long term, especially when the tumor margin dose was 18 Gy. Although GKS can treat multiple tumors in a single session, for HABs associated with VHL disease, GKS faces the dual problems of tumor recurrence or development of a new tumor.

Long-term surgical outcome in 16 patients with thyrotropin pituitary adenoma

2000 ◽  
Vol 93 (2) ◽  
pp. 194-200 ◽  
Author(s):  
Naoko Sanno ◽  
Akira Teramoto ◽  
R. Yoshiyuki Osamura
Keyword(s):  
Cavernous Sinus ◽  
Pituitary Adenomas ◽  
Transsphenoidal Surgery ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Content Type ◽  
Primary Hyperthyroidism ◽  
Fine Print

Object. Thyrotropin-secreting pituitary adenomas are rare lesions of the endocrinological system. Although introduction of a hypersensitive radioimmunoassay for thyrotropin enables the recognition of inappropriate secretion of this hormone, the aforementioned lesions remain uncommon and unfamiliar to most neurosurgeons. It has been reported previously that surgical cure of thyrotropin-secreting adenomas is more difficult than in other functional adenomas because of the large size and invasive features of the former. However, the long-term outcome after surgery has not been well documented. The authors report on a surgical series of 16 patients with thyrotropin adenoma and the results of long-term follow up.Methods. Sixteen patients ages 23 to 62 years (12 women and four men) underwent transsphenoidal removal of thyrotropin adenomas between 1983 and 1999. These patients had the syndrome of inappropriate thyrotropin secretion (SITS) with pituitary mass lesions. Four of the patients had undergone previous subtotal thyroidectomy and/or radioiodine thyroid ablation, and 11 had been treated with antithyroid medication. Radiological investigations demonstrated macroadenomas in 14 patients, and 10 of those had cavernous sinus invasion. Surgical findings showed unusually fibrous and firm tumors in 13 (81.2%) of 16 patients. Preoperative octreotide administration was revealed to be effective for serum thyrotropin reduction as well as tumor shrinkage. Transsphenoidal surgery was performed with no morbidity resulting. Surgical remission was achieved in 10 (62.5%) of 16 patients, and total remission was achieved in 14 patients (87.5%) with a combination of additional radiation or medical therapy. In the other two patients, SITS persisted because of tumor rests in the cavernous sinus. Therefore, radiation and/or antithyroid therapy was administered. In the mean follow-up period of 7.5 years (range 11 months–15.8 years), no recurrence of tumor was observed on magnetic resonance images, whereas recurrence of SITS was found in two patients with no tumor regrowth. In addition, coexistent primary hyperthyroidism was found in two other patients despite remission of SITS after surgery.Conclusions. Transsphenoidal surgery can achieve a good long-term outcome in patients with thyrotropin-secreting pituitary adenomas if surgery is performed before these become larger, invasive tumors. In the authors' experience, thyrotropin-secreting adenomas are fibrous and firm, which makes it difficult to achieve surgical remission. In addition, even satisfactory resection of the tumor sometimes results in recurrence of SITS or hyperthyroid symptoms due to coexistent primary hyperthyroidism. It is emphasized that a careful follow-up review is necessary after surgery, especially in patients with a long preoperative history of hyperthyroidism.

Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery

1995 ◽  
Vol 83 (4) ◽  
pp. 583-589 ◽  
Author(s):  
Leslie N. Sutton ◽  
Patricia T. Molloy ◽  
Heidi Sernyak ◽  
Joel Goldwein ◽  
Peter L. Phillips ◽  
...  
Keyword(s):  
Radical Surgery ◽  
Conservative Surgery ◽  
Low Grade ◽  
Term Outcome ◽  
Term Survival ◽  
Long Term Outcome ◽  
Content Type ◽  
Fine Print

✓ The feasibility of radical surgery for astrocytomas of the optic chiasm/hypothalamus has been reported by several groups. Such surgery carries significant risks, however, including permanent damage to the pituitary gland, optic apparatus, hypothalamic structures, and carotid arteries. The benefits of radical surgery, both in terms of efficacy and toxicity, should, therefore, be evaluated against standard therapy, as is usually done for new chemotherapeutic protocols. To this end, a retrospective review was performed of 33 patients treated at Children's Hospital of Philadelphia between 1976 and 1991 who met criteria that would have made them eligible for radical surgery in many centers today, but were treated with either no surgery or conservative surgery (< 50% resection) or biopsy followed by adjuvant therapy with local radiation therapy (29 patients) and/or chemotherapy with actinomycin-D and vincristine (18 patients). The review encompassed all children with a globular enhancing mass of at least 2 cm in the hypothalamic/chiasmatic region, no evidence of optic nerve involvement or involvement of the optic radiations by computerized tomography or magnetic resonance imaging, and follow up of at least 3 years. All but one patient had tissue confirmation of a low-grade or pilocytic astrocytoma. Thirteen of the patients were 2 years of age or younger at diagnosis. Five individuals died: three of tumor progression, one of acute shunt malfunction, and one of intercurrent infection. The remaining 28 were alive at last follow up, a mean of 10.9 years from diagnosis. Twenty-three surviving patients have functional vision in at least one eye, 12 require no endocrine replacement, and 16 are in or have completed schooling with regular academic requirements. If radical surgery is to become standard care for children with low-grade astrocytomas of the hypothalamic/chiasmatic region, long-term survival and functional outcome will have to equal or surpass those of historical controls who were treated conservatively.

Multiple schwannomas: schwannomatosis or neurofibromatosis type 2?

1998 ◽  
Vol 89 (1) ◽  
pp. 36-41 ◽  
Author(s):  
Matti Tapio Seppälä ◽  
Markku Alarik Sainio ◽  
Matti Jouko Johannes Haltia ◽  
Jaakko Jyri Kinnunen ◽  
Kirsi Hannele Setälä ◽  
...  
Keyword(s):  
Clinical Course ◽  
Positive Family History ◽  
Neurofibromatosis Type ◽  
Magnetic Resonance Images ◽  
Neurofibromatosis Type 2 ◽  
Long Term Outcome ◽  
Content Type ◽  
Fine Print

Object. The aim of this study was to clarify the clinical outcome of schwannomatosis, a rare condition characterized by multiple nonvestibular schwannomas in the absence of meningiomas, intraspinal ependymomas, and other clinical signs of neurofibromatosis type 2 (NF2). Methods. Nine patients with schwannomatosis treated at one institution are presented and their clinical course during a median follow-up time of 9.9 years is discussed. The patients were typically middle-aged at the time of their first operation (median 43.5 years), none had a positive family history of schwannomatosis or NF2, and none showed cutaneous or ocular signs of NF2. On histopathological examination the tumors from the patients with schwannomatosis showed a lobular appearance and frequent Verocay bodies, signs indicating NF2, more often than 20 sporadic schwannomas that were investigated as controls. Two patients died of unrelated causes at 3.2 and 9.9 years, respectively, of follow up. Magnetic resonance images of the head and spine were obtained in seven patients at the end of the follow-up period. New spinal schwannomas were detected in one patient and a residual schwannoma in three. No germline mutations of the NF2 gene were found in these seven patients. Two additional patients originally included in the schwannomatosis group who were 8.6 and 11.7 years old at initial surgery had NF2. One was diagnosed at follow-up review and the other developed a fulminant disease that led to death in 4 years. Conclusions. The clinical course, long-term outcome, and genetic mechanism of schwannomatosis differ from that of NF2.

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

2011 ◽  
Vol 114 (5) ◽  
pp. 1312-1318 ◽  
Author(s):  
Joshua J. Wind ◽  
Kamran D. Bakhtian ◽  
Jennifer A. Sweet ◽  
Gautam U. Mehta ◽  
Jayesh P. Thawani ◽  
...  
Keyword(s):  
Functional Status ◽  
Functional Decline ◽  
Long Term Results ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Von Hippel Lindau ◽  
Presenting Symptoms ◽  
Vhl Disease

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.

Association between a black band on the inner membrane of a chronic subdural hematoma on T2*-weighted magnetic resonance images and enlargement of the hematoma

2003 ◽  
Vol 99 (5) ◽  
pp. 824-830 ◽  
Author(s):  
Toshio Imaizumi ◽  
Yoshifumi Horita ◽  
Toshimi Honma ◽  
Jun Niwa
Keyword(s):  
Surgical Treatment ◽  
Magnetic Resonance ◽  
Chronic Subdural Hematoma ◽  
Magnetic Resonance Images ◽  
Mr Images ◽  
Inner Membrane ◽  
Content Type ◽  
Black Band ◽  
Fine Print

Object. The cause and indication for enlargement of chronic subdural hematomas (CSDHs) have remained unresolved. The authors observed a black band on the inner membrane of a CSDH on T2*-weighted magnetic resonance (MR) images obtained in patients with symptoms. After surgical treatment, the band disappeared. The T2*-weighted sequence of MR imaging is an excellent diagnostic tool for detecting ferromagnetic substances, but it is rarely performed in cases of a CSDH. The authors speculate that the black band may be associated with the development of CSDH. Methods. To investigate how the black band observed on T2*-weighted MR images contributes to the development of a CSDH, 59 lesions in 50 patients with CSDH (41 men and nine women, mean age 70 ± 11 years [range 48–93 years]) were investigated prospectively. The incidence of black bands on the first T2*-weighted MR image obtained in patients with symptomatic CSDH was 97% (31 of 32 CSDHs), which was significantly higher than that associated with asymptomatic CSDH (11% [three of 27 CSDHs], p < 0.001). The black bands associated with symptomatic CSDH disappeared soon after surgical treatment in 31 CSDHs and became fainter in another. In two instances the CSDH recurred with reappearance of the band. Twenty-four of 27 asymptomatic CSDHs had no accompanying black band. Follow-up MR images demonstrated a later formation of bands in two of 24 asymptomatic CSDHs that enlarged to symptomatic size. Logistic regression analysis revealed that the heterogeneity and thickness of the CSDH on computerized tomography scans were independently and significantly associated with the black band. Conclusions. The dynamics of the black band may depend on the enlargement or shrinkage of the CSDH.

scholarly journals Spinal cord hemangioblastomas: significance of intraoperative neurophysiological monitoring for resection and long-term outcome

2017 ◽  
Vol 26 (4) ◽  
pp. 483-493 ◽  
Author(s):  
Sebastian Siller ◽  
Andrea Szelényi ◽  
Lisa Herlitz ◽  
Joerg Christian Tonn ◽  
Stefan Zausinger
Keyword(s):  
Spinal Cord ◽  
Intraoperative Neurophysiological Monitoring ◽  
Neurophysiological Monitoring ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Von Hippel Lindau ◽  
Sensorimotor Deficits ◽  
Vhl Disease

OBJECTIVE Spinal cord hemangioblastomas are rare benign tumors developing either sporadically or as part of von Hippel-Lindau (VHL) disease. Generally, resection is the treatment of choice. However, the significance of intraoperative neurophysiological monitoring (IONM) for resection and postoperative outcome is still controversial. The authors analyzed the surgical and clinical courses of patients who had undergone resection of spinal cord hemangioblastoma, with special attention to preoperative imaging, the use of IONM, and short- and long-term outcomes. METHODS A series of 24 patients (male/female 1:1, lesion sporadic/associated with VHL 2.4:1) who had undergone 26 operations for the resection of 27 spinal cord hemangioblastomas was analyzed. All patients had undergone pre- and postoperative contrast-enhanced MRI. In all cases, microsurgical tumor removal had been performed under continuous IONM of both somatosensory and transcranial motor evoked potentials as well as electromyographic recording. Clinical characteristics, imaging findings, and operative records were retrospectively analyzed. Outcome parameters included short- and long-term status as regards sensorimotor deficits and a questionnaire on general performance, patient satisfaction, and Oswestry Disability Index (ODI) at the end of the follow-up period. The impact of IONM findings on postoperative deficits and outcome parameters as well as risk factors affecting functional prognosis was statistically assessed. RESULTS Preoperative symptoms (mean duration 16.2 ± 22.0 months) included sensory changes (100.0%), pain (66.7%), spinal ataxia (66.7%), motor deficit (41.7%), and bladder/bowel dysfunction (12.5%). Average age at the first operation was 36.8 ± 12.8 years. Most tumors (21 intramedullary, 6 intra- and/or extramedullary) were located dorsally (92.6%) and cervically (77.8%) and were accompanied by peritumoral edema and/or syringomyelia (81.5%). Tumor resection was achieved via laminectomy for 15 tumors, hemilaminectomy for 5, laminoplasty for 6, and interlaminar approach for 1. Gross-total resection was accomplished for 26 tumors (96.3%) with no local tumor recurrence during follow-up. Intraoperative neurophysiological monitoring was nonpathological in 11 operations (42.3%) and pathological in 15 (57.7%). Patients with nonpathological IONM had significantly fewer new sensorimotor deficits (p = 0.005). Long-term follow-up evaluation (mean 7.9 ± 4.0 years postoperatively, 7 patients lost to follow-up) revealed a stable or improved McCormick myelopathy grade in 88.2% of the patients, and 88.2% reported a stable or improved overall outcome according to Odom's criteria. Long-term general performance was excellent with 88.2% having a WHO/Eastern Cooperative Oncology Group (ECOG) Performance Status grade ≤ 1, 76.5% a Karnofsky Performance Scale score ≥ 80, and 70.6% a Barthel Index (BI) of 100. The mean ODI (11.4% ± 12.5%) indicated only minimal disability. There was a significant correlation between pathological IONM findings and a worse long-term status according to the BI and ODI (p = 0.011 and 0.024, respectively). Additionally, VHL disease was a risk factor affecting functional prognosis (p = 0.044). CONCLUSIONS Microsurgical removal of spinal cord hemangioblastomas with IONM facilitates a satisfying long-term outcome for patients. Nonpathological IONM findings are associated with a lower risk of new sensorimotor deficits and correlate with a better overall long-term outcome. von Hippel–Lindau disease is a risk factor for a worse long-term prognosis.

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