Intraspinal hemangioblastomas: analysis of 92 cases in a single institution

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>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 &lt; 1 minute). No complications were observed after 3D CTA; acute paraparesis developed in 1 patient after DSA.</jats:p> <jats:p>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).</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>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.</jats:p></jats:sec>

Conus medullaris ganglioneuroma with syringomyelia radiologically mimicking ependymoma: A case report

“…On occasion, the features of ganglioneuromas may mimic those of myxopapillary ependymomas, which are often located in the conus medullaris and the cauda equina, and are commonly associated with hemorrhages that appear as low-intensity signals on T2WI (9,21). Other spinal lesions, for example hemangioblastomas, which are characterized by small lesions with multi-sectional syringomyelia, are also difficult to differentiate (22). Similarly, in the present case, a contrast-enhanced vessel was identified above the lesion in the T1WI.…”

Incidence and Prognosis of Spinal Hemangioblastoma: A Surveillance Epidemiology and End Results Study

“…Hemangioblastomas of the spinal cord represent approximately 10% of spinal cord tumors [1] . These highly vascular tumors can occur spontaneously or in association with von Hippel-Lindau (VHL) syndrome and can arise in both intramedullary and extramedullary compartments [1] .…”

“…These highly vascular tumors can occur spontaneously or in association with von Hippel-Lindau (VHL) syndrome and can arise in both intramedullary and extramedullary compartments [1] . These are histopathologically benign with low oncologic mortality; however, significant neurological morbidity can occur by their presence in the spinal cord for reason of location or surgical approach.…”

“…The unique pathology and management of spinal hemangioblastoma and prognosis with surgical resection is well described [1,2,5,6,[11][12][13][14][15][16][17][18][19][20][21][22] , there is a marked paucity of epidemiological data. Furthermore, there is no compelling data detailing comparative efficacy of expectant management, surgical resection and radiotherapy in significant population sizes.…”

Imaging diagnosis and the role of endovascular embolization treatment for vascular intraspinal tumors

“…52 Although endovascular embolization can reduce intraoperative blood loss, it is for the most part not routinely performed, as complications were previously reported, including spinal cord swelling, massive venous bleeding, intratumoral hemorrhage, and spinal cord ischemia. 24,29,34,40 Temporary arterial occlusion (TAO) by aneurysm clip placement with concurrent neuromonitoring is a microsurgical technique that helps to differentiate the arteries supplying the tumor from those supplying the healthy spinal cord. 23 To our knowledge, no randomized studies exist comparing the resection of nonmetastatic vascular intraspinal tumors with or without preoperative embolization.…”