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通过后外侧沟入路显微手术切除C1 - C5水平侧位髓内室管膜下瘤:病例报告、手术视频及对诊断和手术挑战的批判性综述

Microsurgical removal of a C1-C5 laterally located intramedullary subependymoma through a postero-lateral sulcus approach: case report, surgical video and critical review of diagnostic and surgical challenges.

作者信息

Mastantuoni Ciro, Bocchetti Antonio, Scala Maria Rosaria, Cioffi Valentina, Corazzelli Giuseppe, Di Colandrea Salvatore, Garofalo Stefania, Fiorentino Francesco, Della Monica Rosa, Chiarotti Lorenzo, de Falco Raffaele

机构信息

Ospedale Santa Maria Delle Grazie, Napoli, Italy.

Ceinge Biotecnologie Avanzate (Italy), Naples, Italy.

出版信息

Eur Spine J. 2025 Aug 12. doi: 10.1007/s00586-025-09246-5.

Abstract

INTRODUCTION

Spinal subependymomas are rare, benign, and slow-growing intramedullary tumors, often misdiagnosed preoperatively due to their radiological resemblance to more common spinal cord neoplasms such as ependymomas and astrocytomas. Given their rarity, the optimal surgical approach and oncological strategy remains debated. While these tumors are histologically benign, obtaining a definitive diagnosis is crucial due to the diagnostic overlap with more aggressive lesions. In this case, a postero-lateral sulcus approach was selected based on the tumor's location and spinal cord rotation, allowing for a safe resection while preserving spinal cord function.

CASE PRESENTATION

A 41-year-old female presented with progressive right arm weakness over two years, followed by gait instability, paresthesia, and severe tetraparesis. Neurological examination revealed right-sided paresis, sensory deficits, and an unsteady gait. MRI demonstrated a T2-hyperintense, T1-hypointense intramedullary lesion from C1 to C5, with an eccentric localization and subpial extension, raising suspicion for an astrocytoma or ependymoma, though a subependymoma remained a consideration. Given the patient's progressive deterioration and the need for a histological diagnosis, surgical intervention was performed. A postero-lateral sulcus approach was chosen due to the lesion's lateral subpial emergence. Intraoperative mapping and neuromonitoring were used to preserve the corticospinal tracts and posterior columns. The tumor was carefully dissected through working windows between the dorsal root entry zones and was completely removed without significant neuromonitoring changes. Histological analysis confirmed the diagnosis of subependymoma, with a low Ki-67 index and no high-grade features. Postoperatively, the patient experienced transient worsening of motor deficits but showed progressive improvement, regaining full right arm function and near-normal gait at follow-up. No adjuvant therapy was required.

CONCLUSION

This case highlights the diagnostic challenges of spinal subependymomas and the role of surgery in securing a definitive diagnosis. The postero-lateral sulcus approach, enabled by the tumor's location and spinal cord rotation, provided a safe and effective approach. Intraoperative mapping and neuromonitoring were essential in minimizing surgical morbidity. Given their benign nature, complete resection is curative, and no additional treatment is required. This report underscores the importance of an individualized surgical strategy in managing rare intramedullary tumors with uncertain preoperative diagnosis.

摘要

引言

脊髓室管膜下瘤是一种罕见的、良性的、生长缓慢的髓内肿瘤,由于其影像学表现与更常见的脊髓肿瘤如室管膜瘤和星形细胞瘤相似,术前常被误诊。鉴于其罕见性,最佳的手术方法和肿瘤治疗策略仍存在争议。虽然这些肿瘤在组织学上是良性的,但由于与更具侵袭性的病变存在诊断重叠,获得明确诊断至关重要。在本病例中,根据肿瘤的位置和脊髓旋转情况选择了后外侧沟入路,在保留脊髓功能的同时实现了安全切除。

病例介绍

一名41岁女性,两年来逐渐出现右臂无力,随后出现步态不稳、感觉异常和严重的四肢轻瘫。神经系统检查发现右侧轻瘫、感觉障碍和步态不稳。磁共振成像(MRI)显示从C1至C5有一个T2高信号、T1低信号的髓内病变,病变偏心定位且有软膜下扩展,怀疑为星形细胞瘤或室管膜瘤,不过室管膜下瘤仍在鉴别考虑范围内。鉴于患者病情逐渐恶化且需要组织学诊断,遂进行了手术干预。由于病变在软膜下向外侧突出,选择了后外侧沟入路。术中采用定位和神经监测以保留皮质脊髓束和后柱。通过背根入区之间的操作窗口仔细分离肿瘤,肿瘤被完全切除,神经监测未出现明显变化。组织学分析确诊为室管膜下瘤,Ki-67指数低,无高级别特征。术后,患者运动功能障碍短暂加重,但随后逐渐改善,随访时右臂功能完全恢复,步态接近正常。无需辅助治疗。

结论

本病例突出了脊髓室管膜下瘤的诊断挑战以及手术在获得明确诊断中的作用。肿瘤位置和脊髓旋转情况使得后外侧沟入路成为一种安全有效的方法。术中定位和神经监测对于将手术并发症降至最低至关重要。鉴于其良性性质,完整切除可治愈,无需额外治疗。本报告强调了个体化手术策略在处理术前诊断不明确的罕见髓内肿瘤中的重要性。

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