Sporadic hemangioblastoma of cauda equina: A case-report and brief literature review

Salvatore D’Oria, David Giraldi, Vincenzo Fanelli, Vincenzo D’Angelo
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Abstract

Hemangioblastomas are rare lesions accounting for 1–5% of all spinal cord tumors and are mostly associated with von Hippel–Lindau syndrome. Localization in the cauda equina is uncommon. In this manuscript we aim to describe a rare case of sporadic intradural extramedullary hemangioblastoma of the cauda equina and present a literature review. A systematic research was performed on Pubmed, MEDLINE, and Google Scholar, using as keywords “spinal hemangioblastoma” and “cauda equina tumors”. The previous literature is integrated by the description of the present case. A 49 year-old female, presented on August 2020 to our institution suffering from claudication neurogena, right sciatica and paraesthesia in right L5 radicular dermatome for more than 3 months. Neurological examination revealed hypoesthesia on right L5 dermatome and weakness of right anterior tibialis muscle. An MRI which showed an intradural mass at L1/2 level and an angiography that showing a nidus of serpiginous vessels inside the lesion. Microsurgical en bloc resection of lesion was performed with adjuvant neurophisological intra operative monitorings. Histological examination provided the diagnosis of hemangioblastoma. After surgery symptoms and neurological impairment gradually improved. A 10 months post-operative MRI showed no residual tumor. Although intradural extramedullary hemangioblastoma of the cauda equina without von Hippel–Lindau syndrome it is a rare pathological entity, this diagnosis must be taken in for cauda equina masses. Preoperative embolization is an option to minimize intraoperative bleeding. Radiosurgery seems to prevent recurrences when the tumor is not completely excised. Complete surgical removal of the lesion is usually possible and lead to a low likelihood of recurrence.

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散发性马尾血管母细胞瘤1例报告并文献复习
血管母细胞瘤是一种罕见的病变,占所有脊髓肿瘤的1-5%,主要与von Hippel-Lindau综合征有关。马尾的定位并不常见。在这篇文章中,我们的目的是描述一个罕见的马尾神经髓外血管母细胞瘤病例,并提出一篇文献综述。在Pubmed、MEDLINE和Google Scholar上进行了一项系统研究,关键词为“脊髓血管母细胞瘤”和“马尾肿瘤”。先前的文献通过对本案的描述而被整合。一名49岁的女性,于2020年8月在我院就诊,患有右L5神经根皮节的跛行神经源、右坐骨神经痛和感觉异常,持续时间超过3个月。神经系统检查显示右侧L5皮节感觉迟钝,右侧胫骨前肌无力。核磁共振成像显示L1/2水平的硬膜内肿块,血管造影术显示病变内有一个带状血管窝。病变的显微外科整体切除术在术中辅助神经病理监测下进行。组织学检查提供了血管母细胞瘤的诊断。术后症状和神经功能损害逐渐好转。术后10个月的MRI显示没有残留肿瘤。尽管没有von Hippel-Lindau综合征的马尾神经硬膜外血管母细胞瘤是一种罕见的病理实体,但必须对马尾肿块进行诊断。术前栓塞是减少术中出血的一种选择。当肿瘤没有完全切除时,放射外科似乎可以防止复发。完全手术切除病变通常是可能的,并且复发的可能性很低。
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