Sporadic Intradural Extramedullary Hemangioblastoma Not Associated with von Hippel-Lindau Syndrome: A Case Report and Literature Review

엄, 미경; 이, 영준; 이, 준우; 이, 규상; 강, 유선; 안, 중모; 강, 흥식

doi:10.3348/jksr.2020.0040

Cited by 3 publications

(5 citation statements)

References 8 publications

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“…On T2WI, paragangliomas are hyperintense and sometimes heterogeneous, owing to subacute blood clots. Serpentine flow voids are a frequent feature attributed to the hypervascularity of the lesion or to congested veins, which are compressed by the mass ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

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Intradural Extramedullary Ependymoma with Hemorrhage: A Case Report

Choi,

Lee,

Paeng

et al. 2023

J Korean Soc Radiol

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In adults, spinal ependymomas are usually found in intramedullary locations. However, intradural extramedullary spinal ependymomas are rare. Additionally, spinal ependymomas usually show iso to hypointensity on T1-weighted images without hemorrhage. Herein, we present a rare case of a 43-year-old female with a pathologically confirmed intradural extramedullary ependymoma that showed hyperintensity on T1-weighted imaging accompanied by hemorrhage.

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Section: Discussionmentioning

confidence: 99%

“…They tend to be hypointense to isointense compared to the intensity of the normal spinal cord on T1WI, and hyperintense on PD-weighted imaging and T2WI. Signal intensity may also be affected by blood products, and calcification may lead to regions of low T2 signal intensity ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

Intradural Extramedullary Ependymoma with Hemorrhage: A Case Report

Choi,

Lee,

Paeng

et al. 2023

J Korean Soc Radiol

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“…Myelopathy may occur with significant tumor growth associated with spinal cord and conus medullaris compromise [5]. Non-contrast and contrast-enhanced MRI are the gold standards for diagnosing hemangioblastomas [8]. In the T1 sequence, the tumor appears isointense, while in T2 it is moderately heterogeneous compared to the spinal cord.…”

Section: Table 1: Overview Of Published Cases With Sporadic Intradura...mentioning

confidence: 99%

“…In the T1 sequence, the tumor appears isointense, while in T2 it is moderately heterogeneous compared to the spinal cord. Generally, gadolinium application leads to intense enhancement of the tumor mass [8].…”

Section: Table 1: Overview Of Published Cases With Sporadic Intradura...mentioning

confidence: 99%

“…The neuroimaging differential diagnosis of an intradural extramedullary tumor in the area of conus medullaris and cauda equina includes a broad variety of spinal intradural lesions, such as nerve sheath tumors (schwannoma or neurofibroma), meningioma, paraganglioma, myxopapillary ependymoma, metastasis, and hemangioblastoma [9,10]. In contrast to intradural metastases, which are usually multiple, the rest of the neoplasms have similar neuroimaging findings and require further differentiation [8] (…”

Section: Table 1: Overview Of Published Cases With Sporadic Intradura...mentioning

confidence: 99%

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Monoradiculopathy Caused by Sporadic Hemangioblastoma of the Conus Medullaris: Case Report and Literature Review

Kehayov¹,

Angelova²,

Batakliev³

et al. 2022

Cureus

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Sporadic spinal extramedullary hemangioblastomas of the conus medullaris are extremely rare. We present the case of a 40-year-old male with symptoms of severe back pain and monoradiculopathy. The magnetic resonance imaging (MRI) revealed an intradural extramedullary tumor attached to the conus medullaris. Total tumor removal was achieved via a typical posterior midline approach through laminectomy of L1 and L2 vertebrae, resulting in complete resolution of the preoperative symptoms. The histological examination was consistent with hemangioblastoma. To the best of our knowledge, this is the fifth case reported in the literature. We performed a brief literature review outlining the mainstay of diagnosis and therapeutic approach to these challenging lesions.

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Integrated surgical intervention for intradural extramedullary hemangioblastoma of the cervical spine: a case report and literature review

Wang,

Zhu,

Chen

et al. 2024

Front. Oncol.

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IntroductionThe incidence of hemangioblastoma is low, constituting only 1-5% of all spinal cord tumors. Specifically, intradural extramedullary hemangioblastoma without Von Hippel-Lindau syndrome represents an exceedingly rare condition.MethodsWe report the first documented case of cervical intradural extramedullary hemangioblastoma in China. A 53-year-old male patient presented with a 3-year history of mild right hemiplegia, segmental muscle strength and sensation impairment, and a positive pyramidal tract sign. MRI showed an abnormal oval signal focus in the intradural and extramedullary region at the C6-C7 vertebral level. Before surgery, angiography was performed to identify the supplying arteries and draining veins. Subsequent interventional therapy achieved over 90% occlusion of blood vessels, creating optimal conditions for complete resection of the spinal tumor.ResultsThe patient demonstrated satisfactory postoperative recovery with significant restoration of sensory and motor functions. Pathological examination showed a significant upregulation of CD31 in tumor cells and a substantial presence of the neuro-specific marker S100 in the tumor stroma, consistent with the diagnostic criteria for spinal hemangioblastoma.ConclusionThe rarity of cervical intradural extramedullary hemangioblastoma without Von Hippel-Lindau syndrome was reaffirmed by a comprehensive review of the existing literature. Complete tumor resection remains the optimal approach for managing this uncommon condition, generally resulting in a favorable prognosis. Traditional open fenestration surgery is linked to elevated risks of bleeding and trauma. Meanwhile, endovascular injection of embolic agents may lead to residual lesions and an increased risk of recurrence. Therefore, we recommend a one-time combined treatment conducted in a hybrid operating room to achieve complete resection and effectively reduce intraoperative bleeding risk. Despite presenting challenges and requiring high proficiency, we still recommend this type of combined surgery as a suitable therapeutic option for such diseases.

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Sporadic Intradural Extramedullary Hemangioblastoma Not Associated with von Hippel-Lindau Syndrome: A Case Report and Literature Review

Cited by 3 publications

References 8 publications

Intradural Extramedullary Ependymoma with Hemorrhage: A Case Report

Intradural Extramedullary Ependymoma with Hemorrhage: A Case Report

Monoradiculopathy Caused by Sporadic Hemangioblastoma of the Conus Medullaris: Case Report and Literature Review

Integrated surgical intervention for intradural extramedullary hemangioblastoma of the cervical spine: a case report and literature review

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