Alvarez Reinier, Mastorakos Panagiotis, Chittiboina Prashant
Neurosurgery Unit for Pituitary and Inheritable Diseases, National Institute of Neurological Disorders and Stroke, Bethesda, MD, United States.
Florida International University Herbert Wertheim College of Medicine, Miami, FL, United States.
Interdiscip Neurosurg. 2021 Mar;23. doi: 10.1016/j.inat.2020.100904. Epub 2020 Sep 10.
Conus 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.
A 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.
Timely 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.
圆锥肿瘤较为罕见，因为大多数脊髓肿瘤发生在颈段和胸段。脊髓血管母细胞瘤占所有髓内脊髓肿瘤的3%-4%，可散发或与冯·希佩尔-林道病相关。文献中仅发表了14例圆锥血管母细胞瘤病例，本文报告第15例。
一名患有冯·希佩尔-林道病的44岁男性，出现双侧下肢无力加重、步态失衡，会阴和生殖器感觉缺失，伴有肛门自主收缩无力。磁共振成像（MRI）显示多个稳定的脊髓肿瘤以及一个6毫米的圆锥血管母细胞瘤，有生长迹象且出现了新的瘤周囊肿。患者接受了顺利的手术切除，术后会阴感觉和括约肌控制有明显改善。
及时切除圆锥肿瘤可缓解症状，防止严重神经功能缺损的进展。精心的显微手术解剖并选择合适的患者，能够安全地切除圆锥肿瘤。
