Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease

Mehta, Gautam U.; Asthagiri, Ashok R.; Bakhtian, Kamran D.; Auh, Sungyoung; Oldfield, Edward H.; Lonser, Russell R.

doi:10.3171/2009.10.spine09592

Cited by 76 publications

(79 citation statements)

References 32 publications

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“…Three months after surgery, her neuropathic pain syndrome started to become considerably aggravated. of these tumors underwent GTR 7,17,19,20,27,34,37,44,[56][57][58]63 with a significant and steady increase of this rate throughout the study period. The corresponding learning curve was evident for the entire department as well as for individual surgeons.…”

Section: Resection Resultsmentioning

confidence: 99%

“…2 gave a figure of 13.9% for ependymomas, Liang et al 35 of 6.2% for cavernomas, Lu and Lawton 36 of 9% for cavernomas, Mehta et al 44 of 4% for angioblastomas, and Van Velthoven et al 59 of 0% for angioblastomas. In this study, rates for permanent morbidity also varied according to histology and were lowest for angioblastomas and cavernomas but highest for ependymomas similar to these literature reports.…”

Section: Permanent Surgical Morbiditymentioning

confidence: 99%

“…In such cases, resections required no separate myelotomy and the tumor could be entered directly. This applied to most angioblastomas, 6,38,44,57 some cavernomas, 35,36,55 and a few astrocytomas. Whenever the spinal cord had covered the intramedullary tumor completely, sensory functions suffered permanently to some degree.…”

Section: Postoperative Sensory Dysfunctionsmentioning

confidence: 99%

“…In VHL, a recurrence may develop anytime and anywhere in the spinal canal. 44 For patients without VHL, however, no recurrence should be observed after a complete resection. The 2 recurrences in this study could be attributed to an incomplete resection in one case and an extramedullary recurrence on the ventral side of the cord in the other after resection of a giant angioblastoma penetrating the cord completely.…”

Section: 53mentioning

confidence: 99%

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Treatment of intramedullary tumors: analysis of surgical morbidity and long-term results

Klekamp

2013

SPI

109

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Object Surgery of intramedullary tumors is established as the treatment of choice for these challenging lesions. This study presents a detailed analysis of risk factors for surgical morbidity and data on long-term results for intramedullary tumors. Methods Among 1317 patients with tumors of the spinal canal treated between 1980 and 2012, 278 patients with intramedullary tumors are presented. A total of 225 of these patients underwent 246 operations for treatment of 250 tumors. The mean patient age was 41 ± 17 years (range 3 weeks to 83 years). Patients underwent follow-up through outpatient visits and questionnaires with a mean follow-up of 41 ± 53 months. Tumors were subdivided into 3 groups: displacing tumors (Type A, n = 162), infiltrating tumors (Type B, n = 80), and nonproliferating tumors (Type C, n = 8). A gross-total resection (GTR) was attempted for every tumor except for Type C lipomas. Participating surgeons were divided into 3 groups according to the number of operations they performed. Short-term results were determined for individual symptoms and the modified McCormick Scale, whereas tumor recurrence rates were calculated with Kaplan-Meier statistics. Results Overall, 83.3% of Type A tumors underwent GTR compared with 22.5% of Type B and none in Type C. Gross-total resection rates increased throughout the study period and correlated significantly with surgical experience. A worsened neurological state after surgery was seen in 61% of patients. This deterioration was transient in 41.5% and was a common observation after GTR. Permanent morbidity (19.5%) was lowest after GTR and correlated significantly with surgical experience and the preoperative neurological state. Further analysis showed that patients with tumors of thoracic levels, tumor hemorrhages, and malignant and recurrent tumors were at a higher risk for permanent morbidity. In the long term, tumor recurrence rates for ependymomas and benign astrocytomas correlated significantly with the amount of resection. Long-term morbidity affected 3.7% with a postoperative myelopathy related to cord tethering at the level of surgery and 21.9% in form of neuropathic pain syndromes. The rate of postsurgical cord tethering could be lowered significantly by using pia sutures after tumor resection. Neuropathic pain syndromes were more common after surgery for tumors with associated syringomyelia or those located in the cervical cord. Conclusions Intramedullary tumors should be surgically treated as soon as neurological symptoms appear. Gross-total resection is possible for the majority of benign pathologies. Cervical tumors are associated with higher GTR and lower permanent morbidity rates compared with thoracic tumors. Surgery on intramedullary tumors should be performed by neurosurgeons who deal with these lesions on a regular basis as considerable experience is required to achieve high GTR rates and to limit rates of permanent morbidity.

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Section: Resection Resultsmentioning

confidence: 99%

Section: Permanent Surgical Morbiditymentioning

confidence: 99%

Section: Postoperative Sensory Dysfunctionsmentioning

confidence: 99%

Section: 53mentioning

confidence: 99%

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Treatment of intramedullary tumors: analysis of surgical morbidity and long-term results

Klekamp

2013

SPI

109

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“…Tumor volume was determined using the following equation: greatest anteroposterior dimension × greatest mediolateral dimension × greatest craniocaudal dimension × 0.5. 17 Gross-total resection was defined as complete tumor removal according to operative microscopic findings and postoperative contrast-enhanced T1-weighted MR images.…”

Section: Imaging Evaluationmentioning

confidence: 99%

Intraspinal hemangioblastomas: analysis of 92 cases in a single institution

Deng¹,

Wang

Wu³

et al. 2014

SPI

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Object Intraspinal hemangioblastomas are relatively uncommon benign tumors. The surgical strategies remain controversial, and the risk factors with regard to clinical outcome are unclear. The purpose of this study was to analyze the clinical characteristics, imaging findings, surgical strategies, and functional outcomes associated with intraspinal hemangioblastomas. Methods A series of 92 patients who underwent 102 operations for resection of 116 intraspinal hemangioblastomas at a single institution during 2007–2011 were consecutively enrolled in this study. Of these, 60 patients (65.2%) had sporadic hemangioblastomas and 32 (34.8%) had von Hippel-Lindau disease. Preoperatively, 13 patients underwent digital subtraction angiography (DSA), 15 patients underwent 3D CT angiography (3D CTA), and none underwent preoperative embolization. Clinical characteristics, imaging findings, and operative records were analyzed. The advantages and disadvantages of DSA and 3D CTA were compared. For identification of risk factors that affect prognosis, logistic analysis was performed. Results The male/female patient ratio was 1.8:1.0 (59 male and 33 female patients). Of the tumors, 41% were intramedullary, 37% were intramedullary-extramedullary, and 22% were primarily extramedullary. Three-dimensional CTA and DSA did not differ significantly in the ability to identify the feeding arteries (p = 1.000) and image qualities (p = 0.367). However, compared with 3D CTA, the effective x-ray dose of spinal DSA was 2.73 times higher and the mean amount of contrast media injected was 1.88 times higher. Spinal DSA was more time consuming (mean 120 minutes) than 3D CTA (scanning time < 1 minute). No complications were observed after 3D CTA; acute paraparesis developed in 1 patient after DSA. Gross-total resection was achieved for 109 tumors (94.0%), and resection was subtotal for 7 tumors. Mean duration of follow-up was 50 months (range 24–78 months). At the most recent follow-up visit, the functional outcome was improved for 38 patients (41.3%), remained stable for 40 (43.5%), and deteriorated for 14 (15.2%). Logistic analysis showed that subtotal resection was a risk factor affecting prognosis (p = 0.003, OR 12.833, 95% CI 2.429–67.806). Conclusions The authors' study suggests that safe and effective treatment of intraspinal hemangioblastomas can be achieved for most patients, even without preoperative embolization. Gross-total resection, when safe to perform, leads to better outcomes. Compared with spinal DSA, 3D CTA is a promising technique because it is noninvasive, takes less time to perform, requires lower x-ray doses and less contrast media, results in fewer complications, and offers high accuracy for delineating the feeding arteries.

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Hemangioblastoma

Singh

Zagzag

2014

Molecular Pathology Library

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Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease

Cited by 76 publications

References 32 publications

Treatment of intramedullary tumors: analysis of surgical morbidity and long-term results

Treatment of intramedullary tumors: analysis of surgical morbidity and long-term results

Intraspinal hemangioblastomas: analysis of 92 cases in a single institution

Hemangioblastoma

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