Habib Ahmed, Deng Hansen, Hameed N U Farrukh, Kulich Scott, Zinn Pascal
Department of Neurosurgery, University of Pittsburgh, Pittsburgh, Pennsylvania, United States.
Department of Pathology, University of Pittsburgh, Pittsburgh, Pennsylvania, United States.
Surg Neurol Int. 2024 Jul 12;15:237. doi: 10.25259/SNI_317_2024. eCollection 2024.
Ependymomas, rare glial brain tumors, account for <5% of all brain tumors. Interestingly, over 60% of ependymomas occur in the spinal cord of adults, including those originating from the filum terminale, while the rest are found within the brain. The World Health Organization (WHO) categorizes ependymomas into three grades: subependymomas and myxopapillary ependymomas ([MEPNs]; WHO grade I), classic ependymomas (WHO grade II), and anaplastic ependymomas (WHO grade III). Spinal ependymomas generally exhibit a more favorable prognosis compared to their intracranial counterparts and are primarily treated through gross total resection, which is considered the most effective surgical approach. As such, they are recognized as a distinct clinical entity that demands tailored management strategies. MEPNs, which constitute 13% of ependymomas, typically occur in the cauda equina and sometimes extend into the conus medullaris. Most other spinal ependymomas are of the classic type and predominantly arise in the cervical and thoracic regions of the spine. The mean age at diagnosis is 45 years of age. While prognosis varies based on molecular subtypes, complete resection is associated with improved survival.
Here, we demonstrate the technical nuances to safely achieve gross total resection of a giant spinal ependymoma in a 29-year-old female with a medical history notable for sept-optic dysplasia, and panhypopituitarism. The patient presented with progressive neck pain, upper and lower extremity weakness, and numbness for 1 year. On physical examination, she demonstrated mild weakness in her left arm. The preoperative magnetic resonance imaging revealed a cervicothoracic intramedullary mass extending from C4 to T2 with an associated syrinx at C4. Under intraoperative neural monitoring (somatosensory evoked potentials, motor-evoked potentials, and epidural direct wave recordings), the patient underwent a C4 - T2 laminectomy. In addition, spinal ultrasonography helped differentiate solid tumor mass from syrinx formation, thus guiding the focus and extent of the decompression .
Gross total resection was achieved; at 18 postoperative months, the patient had mild residual motor deficit. The pathological evaluation revealed a WHO grade II ependymoma. Subsequent sequential enhanced MR studies at 3, 6, and 12 months confirmed no tumor recurrence.
室管膜瘤是一种罕见的胶质脑肿瘤,占所有脑肿瘤的比例不到5%。有趣的是,超过60%的室管膜瘤发生在成人脊髓,包括起源于终丝的肿瘤,其余的则发生在脑内。世界卫生组织(WHO)将室管膜瘤分为三个级别:室管膜下瘤和黏液乳头型室管膜瘤([MEPNs];WHO I级)、经典型室管膜瘤(WHO II级)和间变性室管膜瘤(WHO III级)。与颅内室管膜瘤相比,脊髓室管膜瘤的预后通常较好,主要通过全切除治疗,这被认为是最有效的手术方法。因此,它们被认为是一种需要量身定制管理策略的独特临床实体。MEPNs占室管膜瘤的13%,通常发生在马尾,有时会延伸至脊髓圆锥。大多数其他脊髓室管膜瘤为经典型,主要发生在脊柱的颈段和胸段。诊断时的平均年龄为45岁。虽然预后因分子亚型而异,但完全切除与生存率提高相关。
在此,我们展示了在一名有视隔发育不良和全垂体功能减退病史的29岁女性中安全实现巨大脊髓室管膜瘤全切除的技术细节。患者出现进行性颈部疼痛、上肢和下肢无力及麻木1年。体格检查时,她左侧手臂有轻度无力。术前磁共振成像显示一个从C4延伸至T2的颈胸段髓内肿块,C4处伴有空洞。在术中神经监测(体感诱发电位、运动诱发电位和硬膜外直接波记录)下,患者接受了C4 - T2椎板切除术。此外,脊髓超声有助于区分实体肿瘤肿块和空洞形成,从而指导减压的重点和范围。
实现了全切除;术后18个月,患者有轻度残留运动功能障碍。病理评估显示为WHO II级室管膜瘤。随后在3个月、6个月和12个月进行的连续增强磁共振研究证实无肿瘤复发。