Characterization of Hemangioblastomas of Spinal Nerves

Gläsker, Sven; Berlis, Ansgar; Pagenstecher, Axel; Vougioukas, Vassilios I.; Velthoven, Vera Van

doi:10.1227/01.neu.0000153909.70381.c8

Cited by 45 publications

(40 citation statements)

References 16 publications

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“…They are usually found in young patients and become clinically evident if producing compression syndromes [30,31]. Hemangioblastomas are even rarer and found in the spinal nerve or nerve roots at their exit out of the vertebral column [32]. They are seen more frequently in von Hippel Lindau's disease together with similar lesions in the CNS.…”

Section: Intraneural Hemangioma and Hemangioblastomamentioning

confidence: 99%

High-resolution ultrasound of peripheral neurogenic tumors

et al. 2007

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Peripheral nerve tumors are not frequent, but due to their association with a nerve they are somewhat special. They may be encountered incidentally during evaluation of a soft-tissue mass or when a nerve lesion is clinically suspected and the recognition of such a lesion and its differential diagnosis is key for successful therapy and patient prognosis. As sonography is often the first line modality in the work-up of a soft-tissue mass, the sonographer should be aware of the typical features of such lesions in order to arrive at the correct diagnosis, and this article tries to give an overview of the histological subtypes of peripheral nerve tumors and their sonographic characteristics.

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Section: Intraneural Hemangioma and Hemangioblastomamentioning

confidence: 99%

High-resolution ultrasound of peripheral neurogenic tumors

et al. 2007

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“…4,7,10,22,24,36) Hemangioblastomas can present as sporadic, isolated lesions or as a subset of CNS lesions in von Hippel-Lindau (VHL) syndrome. 1,7,24,25,33,36) The clinical characteristics of patients with sporadic CNS hemangioblastomas differ from those of patients with VHL syndrome-associated lesions.…”

Section: Introductionmentioning

confidence: 99%

Comparative Analysis of Spinal Hemangioblastomas in Sporadic Disease and Von Hippel-Lindau Syndrome

Takai

Taniguchi

Takahashi

et al. 2010

Neurol. Med. Chir.(Tokyo)

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The clinical differences were evaluated in spinal hemangioblastoma between patients with sporadic disease and patients with von Hippel-Lindau (VHL) syndrome. The distribution of hemangioblastomas in the central nervous system was investigated in 56 patients treated between 1988 and 2008 at the University of Tokyo Hospital. The characteristics of spinal hemangioblastomas were compared in 35 patients including 17 with sporadic disease and 18 with VHL syndrome treated between 1988 and 2008 at our hospital and three affiliated institutions. Spinal hemangioblastomas were much more prevalent in patients with VHL syndrome (88.2%) than in patients with sporadic disease (20.5%, p º 0.001). Spinal hemangioblastomas associated with VHL syndrome were diagnosed a decade earlier (p ＝ 0.007) and were associated with less severe neurological symptoms than sporadic lesions (p ＝ 0.004). Most patients with sporadic disease had a single lesion at the cervical or thoracic level, whereas patients with VHL syndrome had multiple lesions at all spinal levels (p ＝ 0.04). Patients with sporadic disease exhibited significant improvement in postoperative neurological status (p ＝ 0.02), whereas patients with VHL syndrome had no change in status (p ＝ 1.00). Number of removed lesions (p ＝ 0.03) and lower spinal cord lesions (p ＝ 0.05) were associated with poor surgical outcome. Tumor recurrence was correlated with partial removal of lesions (p ＝ 0.05). One third of patients with VHL syndrome developed new lesions every 2 years. The major finding of this study is that the incidence of spinal hemangioblastoma, distributed through all spinal levels, may be as high as 88% in patients with VHL syndrome, which is much greater than previously reported.

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“…11) Furthermore, the tumor usually affects only the dorsal root fascicles. 1,10,12,19) However, in our patient, the entire L2 nerve root was fanning out and completely encased by the tumor, and the tumor was tightly attached to the surrounding tissue due to bleeding at the onset. We were forced to resect the entire L2 nerve root to accomplish extracapsular removal, but the patient's postoperative neurological symptoms were minimal as in previous cases (Table 2), because the function of the fanning origin nerve might be compensated by other nerves during tumor growth, as occasionally experienced in schwannoma.…”

Section: Discussionmentioning

confidence: 60%

“…12) Intra-extradural dumbbell-shaped hemangioblastoma is extremely rare; previous reports of this tumor are listed in Table 2. 1,4,7,10,12,19,23,26) Hemangioblastomas are certainly a diagnostic challenge and may be preoperatively misdiagnosed as metastases, schwannomas, or neurofibromas, all of which should be treated by distinct strategies. It is imperative to identify hemangioblastomas preoperatively because these tumors tend to bleed significantly at surgery because of their vascular nature.…”

Section: Discussionmentioning

confidence: 99%

Intra-extradural Dumbbell-Shaped Hemangioblastoma Manifesting as Subarachnoid Hemorrhage in the Cauda Equina

Nishimura

Hara

Natsume

et al. 2012

Neurol. Med. Chir.(Tokyo)

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A 56-year-old man presented with a rare intra-extradural dumbbell-shaped hemangioblastoma in the lumbar spine associated with von Hippel-Lindau (VHL) disease manifesting as subarachnoid hemorrhage. The tumor, which was exiting at the right L2-3 intervertebral foramen, was removed successfully by total facetectomy and posterior spinal fusion at the L2-3 segment. Nine years later, a recurrent tumor due to VHL was also totally removed with minimal neurological complications. Accurate diagnosis of this vascular-rich lesion is essential for developing an adequate surgical strategy. The dumbbell-shaped tumor requires total facetectomy and spinal reconstruction, and care should be taken to preserve the entire nerve root origin by only identifying the affected nerve fascicles at the origin, if possible. Postoperative adhesion must be minimized for second surgery in patients with genetic disease such as VHL, who are likely to suffer recurrence.

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Characterization of Hemangioblastomas of Spinal Nerves

Cited by 45 publications

References 16 publications

High-resolution ultrasound of peripheral neurogenic tumors

High-resolution ultrasound of peripheral neurogenic tumors

Comparative Analysis of Spinal Hemangioblastomas in Sporadic Disease and Von Hippel-Lindau Syndrome

Intra-extradural Dumbbell-Shaped Hemangioblastoma Manifesting as Subarachnoid Hemorrhage in the Cauda Equina

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