Nodal Samuel, Khalafallah Adham M, Yunga Tigre Joseph, Gultekinm Sakir Humayun, Sanghera Bhavjeet Singh, Levi Allan D, Burks S Shelby
Department of Neurological Surgery, University of Miami Miller School of Medicine, Miami, United States.
Department of Pathology, University of Miami Miller School of Medicine, Miami, United States.
Surg Neurol Int. 2025 May 16;16:188. doi: 10.25259/SNI_267_2025. eCollection 2025.
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.
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.
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.
神经鞘瘤与脑膜瘤一样，是硬脊膜内髓外肿瘤最常见的类型。它们是罕见的、通常为良性的肿瘤，起源于施万细胞。这些肿瘤的症状和体征可能包括背部/神经根性疼痛、局灶性运动和/或感觉障碍以及肠道/膀胱功能障碍。对该病变进行病理确诊并将其与其他良性病变（即神经纤维瘤、脂肪瘤、神经节囊肿、脑膜瘤和巨细胞瘤）和/或罕见的恶性病变（即恶性外周神经鞘瘤和肉瘤）相鉴别至关重要。上皮样神经鞘瘤/良性上皮样神经鞘瘤（BES）是神经鞘瘤的一种良性、罕见的组织学亚型，类似于上皮细胞，缺乏诸如Antoni A区和Antoni B区等典型特征。
一名29岁女性，出现严重、持续、尖锐的背部疼痛（即疼痛程度为5 - 10/10）达1年，疼痛沿左下肢放射。她还经历了双侧腿部麻木和刺痛，以及臀部和右下肢无力。双氯芬酸未能缓解任何疼痛。值得注意的是，尽管进行了物理治疗，症状仍有进展。她的神经系统检查显示左侧胫前肌和拇长伸肌分布区域有轻度无力（4+/5）。磁共振成像显示L2水平有一个不均匀强化的硬脊膜内髓外环形肿块，导致马尾神经受压。患者接受了L1 - L2椎板切除术，这是推荐的肿瘤切除手术。病理检查显示该病变为BES；上皮细胞S - 100染色呈阳性。术后三个月，患者无症状。
BES是神经鞘瘤的一种罕见亚型。患有这些病变的患者应进行肿瘤全切以获得最佳的长期术后效果。
