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成人后颅窝和脊髓血管母细胞瘤的外科治疗及预后:6 例病例系列及文献复习。

Surgical management and outcome of adult posterior cranial fossa and spinal hemangioblastoma: a 6-case series and literature review.

机构信息

Department of Neurosurgery, Sestre Milosrdnice University Hospital Center , Zagreb, Croatia.

School of Dental Medicine and Health, J.J. Strossmayer University of Osijek , Osijek, Croatia.

出版信息

Neurol Res. 2020 Dec;42(12):1010-1017. doi: 10.1080/01616412.2020.1796382. Epub 2020 Jul 22.

Abstract

OBJECTIVE

Hemangioblastomas of the posterior cranial fossa and spinal cord in adults are excessively vascularized, well-differentiated, and scarce tumors with no metastatic potential. This paper discusses its surgical management and outcome, pointing out their morphological, radiological, and histopathological aspects. This report based on a personal series of six patients and on a literature review.

METHODS

A single-institution personal 6-case series of adult patients diagnosed and operated on by a senior neurosurgeon (KIA) due to posterior cranial fossa or spinal cord hemangioblastoma was analyzed. For easier understanding of hemangioblastoma, we have classified them into four different types.

RESULTS

The tumors, which were all surgically treated, were located in the posterior cranial fossa in five patients (4 cerebellar, 1 brain stem) and intramedullary in the thoracic spinal cord in one patient. All patients successfully recovered neurologically after a complete tumor resection, having no post-operative neurological deficit or other complications.

CONCLUSION

Surgical management of cerebellar and spinal cord hemangioblastoma in adults is highly dependent on its morphological features, as well as on microsurgical technique applied. Since huge differences exist between the cystic/nodular tumor type (Type 1) and the solid type and its two additional variants (Types 2-4), morphology is the most important consideration when deciding surgical approach. Despite significant morphological differences among different subtypes of hemangioblastomas, their histology appears to be relatively similar. Nonetheless, a meticulous and refined surgical technique has to be utilized to achieve a successful outcome.

摘要

目的

成人后颅窝和脊髓的血管母细胞瘤是高度血管化、分化良好、罕见且无转移潜能的肿瘤。本文讨论了其手术治疗及预后,指出了其形态学、影像学和组织病理学特征。本报告基于作者个人的 6 例患者系列和文献复习。

方法

作者分析了由一位资深神经外科医生(KIA)诊断和治疗的 6 例成人后颅窝或脊髓血管母细胞瘤患者的单中心个人病例系列。为了更方便理解血管母细胞瘤,我们将其分为 4 种不同类型。

结果

所有肿瘤均通过手术治疗,5 例位于后颅窝(4 例小脑,1 例脑干),1 例位于胸髓内。所有患者在完全切除肿瘤后均成功恢复神经功能,无术后神经功能缺损或其他并发症。

结论

成人小脑和脊髓血管母细胞瘤的手术治疗高度依赖于其形态特征和应用的显微外科技术。由于囊性/结节型肿瘤(1 型)与实性型及其两个附加变体(2-4 型)之间存在巨大差异,因此在决定手术方法时形态是最重要的考虑因素。尽管不同亚型的血管母细胞瘤在形态上存在显著差异,但它们的组织学似乎相对相似。然而,为了获得成功的结果,必须采用细致和精细的手术技术。

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