Resection of a conus medullaris hemangioblastoma: Case report.
Resection of a conus medullaris hemangioblastoma: Case report.
复制标题
髓圆锥血管母细胞瘤切除术：病例报告。
DOI：
10.1016/j.inat.2020.100904
复制
发表时间：
2021
期刊：
影响因子：
--
通讯作者：
Chittiboina,Prashant
中科院分区：
文献类型：
--
作者：
Alvarez,Reinier;Mastorakos,Panagiotis;Chittiboina,Prashant
背景脊髓圆锥肿瘤很少见，因为大多数脊髓肿瘤发生在颈部和胸部区域。脊髓血管母细胞瘤占所有髓内脊髓肿瘤的 3%–4%，可以是散发性的或与 von Hippel-Lindau 病相关。文献中发表的圆锥髓血管母细胞瘤病例仅有14例，本文介绍第15例。 病例描述一名44岁男性，患有von Hippel Lindau病，表现为双下肢无力加重，步态不平衡，会阴和生殖器感觉缺失，肛门自主收缩无力。 MRI 显示多个稳定的脊柱肿瘤和一个 6 毫米的圆锥形血管母细胞瘤，并有生长和新的肿瘤周围囊肿。该患者接受了简单的手术切除，术后会阴感觉和括约肌控制明显改善。结论及时切除髓圆锥肿瘤可以缓解症状并防止破坏性神经功能缺损的进展。仔细的显微外科解剖和适当的患者选择可以安全切除髓圆锥肿瘤。
BackgroundConus medullaris tumors are rare, as the majority of all spinal cord tumors occur in the cervical and thoracic regions. Hemangioblastomas of the spinal cord account for 3%–4% of all intramedullary spinal cord tumors and can be sporadic or associated with von Hippel-Lindau disease. There are only fourteen cases of conus medullaris hemangioblastomas published in the literature, herein we present the fifteenth.Case descriptionA 44-year old male with von Hippel Lindau disease presented with worsening bilateral lower extremity weakness, gait imbalance as well as absent perineal and genital sensation with weak voluntary anal contraction. MRI demonstrated multiple stable spinal tumors and a 6 mm conus medullaris hemangioblastoma with growth and a new peri-tumoral cyst. The patient underwent uncomplicated surgical resection with appreciable improvement in perineal sensation and sphincteric control during post-operative course.ConclusionsTimely resection of conus medullaris tumors can provide symptomatic relief and prevent the progression of devastating neurological deficits. Careful microsurgical dissection with appropriate patient selection allows for safe resection of conus medullaris tumors.