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.

中文翻译：

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成人后颅窝和脊髓血管母细胞瘤的手术治疗和结果：6 例系列和文献综述。

摘要

客观的

成人后颅窝和脊髓的血管母细胞瘤是过度血管化、分化良好且没有转移潜能的稀有肿瘤。本文讨论了其手术管理和结果，指出了它们的形态学、放射学和组织病理学方面。本报告基于 6 名患者的个人系列和文献综述。

方法

对由高级神经外科医生 (KIA) 诊断和手术的单一机构个人 6 例成人患者系列进行分析，这些患者是由于后颅窝或脊髓血管母细胞瘤。为了更容易理解血管母细胞瘤，我们将它们分为四种不同的类型。

结果

5名患者（4个小脑，1个脑干）的肿瘤位于后颅窝，1名患者位于胸脊髓髓内，均通过手术治疗。所有患者在完全切除肿瘤后神经系统均成功恢复，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以及所应用的显微外科技术。由于囊性/结节性肿瘤类型（1 型）和实体型及其两种其他变体（2-4 型）之间存在巨大差异，因此在决定手术入路时，形态学是最重要的考虑因素。尽管血管母细胞瘤的不同亚型之间存在显着的形态学差异，但它们的组织学似乎相对相似。尽管如此，必须使用细致和精致的手术技术才能获得成功的结果。