切除一罕见的腰椎上皮样神经鞘瘤。

Surgical neurology international Pub Date : 2025-05-16 eCollection Date: 2025-01-01 DOI:10.25259/SNI_267_2025
Samuel Nodal, Adham M Khalafallah, Joseph Yunga Tigre, Sakir Humayun Gultekinm, Bhavjeet Singh Sanghera, Allan D Levi, S Shelby Burks
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引用次数: 0

摘要

背景:神经鞘瘤和脑膜瘤是最常见的硬膜内髓外肿瘤。它们是罕见的,典型的良性肿瘤,起源于雪旺细胞。这些肿瘤的症状和体征可能包括背部/神经根痛,局灶性运动和/或感觉缺陷,以及肠/膀胱功能障碍。必须对该病变进行病理确认,并将其与其他良性病变(如神经纤维瘤、脂肪瘤、神经节囊肿、脑膜瘤和巨细胞瘤)和/或罕见恶性病变(如恶性周围神经鞘肿瘤和肉瘤)区分开来。上皮样神经鞘瘤/良性上皮样神经鞘瘤(BES)是一种良性、罕见的组织学亚型神经鞘瘤,与上皮细胞相似,缺乏Antoni a区和Antoni B区等典型特征。病例描述:一名29岁女性,表现为1年的严重,持续,尖锐的背部疼痛(即5-10/10),并向左下肢放射。她还经历了双侧腿麻木和刺痛,以及臀部和右下肢无力。双氯芬酸不能缓解疼痛。值得注意的是,尽管使用了物理治疗,症状仍在恶化。神经学检查显示左侧胫骨前肌和拇长伸肌分布轻度无力(4+/5)。磁共振成像显示在L2水平有一个不均匀增强的环形硬膜内髓外肿块,导致马尾受压。患者接受L1-L2椎板切除术,推荐用于肿瘤切除。病理证实病变为BES;上皮细胞S-100染色阳性。术后3个月,患者无症状。结论:BES是罕见的神经鞘瘤亚型。有这些病变的患者应行肿瘤全切除术以获得最佳的术后长期预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Resection of a rare lumbar epithelioid schwannoma.

Background: Schwannomas, along with meningiomas, constitute the most common type of intradural extramedullary tumors. They are rare, typically benign tumors that originate from Schwann cells. Symptoms and signs attributed to these tumors may include back/radicular pain, focal motor and/or sensory deficits, and bowel/bladder dysfunction. It is essential to obtain pathological confirmation of this lesion and to differentiate it from other benign (i.e., neurofibromas, lipomas, ganglion cysts, meningiomas, and giant cell tumors), and/ or infrequently malignant lesions (i.e., malignant peripheral nerve sheath tumors, and sarcomas). Epithelioid Schwannomas/Benign Epithelioid Schwannomas (BES) is a benign, rare histologic subtype of schwannoma that resembles epithelial cells and lacks classic features such as Antoni A and Antoni B areas.

Case description: A 29-year-old female presented with 1 year of severe, constant, sharp back pain (i.e., 5-10/10) that radiated down the left lower extremity. She also experienced bilateral leg numbness and tingling, along with weakness in the hips and right lower extremity. Diclofenac failed to provide any pain relief. Notably, symptoms progressed despite the utilization of physical therapy. Her neurological examination showed mild weakness (4+/5) in the left tibialis anterior and extensor hallucis longus distributions. The magnetic resonance imaging revealed a heterogeneously enhancing circumferential intradural extramedullary mass at the L2 level that caused cauda equina compression. The patient underwent an L1-L2 laminectomy, which was recommended for tumor resection. Pathologically, the lesion proved to be a BES; the epithelial cells stained positive for S-100. Three months postoperatively, the patient is asymptomatic.

Conclusion: BES is a rare subtype of schwannoma. Patients with these lesions should undergo gross total tumor resection to achieve the best long-term postoperative outcomes.

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