Wassef Catherine E, Holloway Melissa R, Silberstein Howard
Neurosurgery, University of Rochester Medical Center, Rochester, USA.
Cureus. 2023 Jan 20;15(1):e34017. doi: 10.7759/cureus.34017. eCollection 2023 Jan.
Spinal ependymomas are the most common intramedullary spinal tumor, with a large proportion containing a small intratumoral cyst. Although the signal intensity varies, spinal ependymomas are generally well-demarcated, are not associated with a pre-syrinx, and do not extend above the foramen magnum. Our case demonstrates unique radiographic findings of a cervical ependymoma with a staged approach to diagnosis and resection. The patient is a 19-year-old female who presented with a three-year history of neck pain, progressive arm and leg weakness, falls, and functional decline. MRI revealed an expansile dorsal and centrally located T2 hypointense cervical lesion with a large intratumoral cyst extending from the foramen magnum to the C7 pedicle. Contrasted T1 scans showed an irregular enhancement pattern along the superior tumoral border down to the C3 pedicle. She underwent a C1 laminectomy for open biopsy and cysto-subarachnoid shunt. Postoperative MRI revealed a well-demarcated enhancing mass extending from the foramen magnum to C2. Pathology revealed Grade II ependymoma. She underwent an occipital to C3 laminectomy with gross total resection. Postoperatively she experienced weakness and orthostatic hypotension that improved remarkably upon discharge. Initial imaging was concerning for a higher-grade tumor, with holocervical cord involvement and cervical kyphosis. Given concern for grade and possible extensive C1-7 laminectomy and fusion for resection, a smaller surgery involving drainage of the cyst and biopsy was performed. Postoperative MRI revealed regression of the pre-syrinx, improved tumoral definition, and improvement of cervical kyphosis. This staged approach spared the patient unnecessary surgical intervention such as extensive laminectomy and fusion. We conclude that in cases of a large intratumoral cyst in an extensive intramedullary spinal cord lesion, open biopsy and drainage followed by resection in a staged fashion should be considered. Radiographic changes from the first procedure may affect the surgical approach for ultimate resection.
脊髓室管膜瘤是最常见的髓内脊髓肿瘤,很大一部分含有小的瘤内囊肿。尽管信号强度有所不同,但脊髓室管膜瘤通常边界清晰,与空洞前状态无关,且不延伸至枕骨大孔上方。我们的病例展示了一例颈段室管膜瘤独特的影像学表现以及诊断和切除的分期方法。患者为一名19岁女性,有三年颈部疼痛、进行性手臂和腿部无力、跌倒及功能衰退病史。磁共振成像(MRI)显示一个膨胀性的、位于背侧且中心部位的T2低信号颈段病变,伴有一个从枕骨大孔延伸至C7椎弓根的大瘤内囊肿。增强T1扫描显示沿肿瘤上缘直至C3椎弓根有不规则强化模式。她接受了C1椎板切除术以进行开放活检和囊肿 - 蛛网膜下腔分流术。术后MRI显示一个边界清晰的强化肿块,从枕骨大孔延伸至C2。病理显示为II级室管膜瘤。她接受了枕骨至C3椎板切除术并进行了全切。术后她出现了无力和体位性低血压,出院时明显改善。最初的影像学表现提示可能为高级别肿瘤,累及全颈髓并伴有颈椎后凸。鉴于对肿瘤分级的担忧以及可能需要进行广泛的C1 - 7椎板切除术和融合术以进行切除,遂先进行了一个较小的手术,包括囊肿引流和活检。术后MRI显示空洞前状态消退、肿瘤边界改善以及颈椎后凸改善。这种分期方法使患者避免了不必要的手术干预,如广泛的椎板切除术和融合术。我们得出结论,对于广泛的髓内脊髓病变伴有大瘤内囊肿的病例,应考虑先进行开放活检和引流,然后分期进行切除。首次手术的影像学变化可能会影响最终切除的手术方式。