Department of Orthopaedic Surgery, Keio University School of Medicine, Tokyo, 160-8582, Japan.
Department of Pathology, Keio University School of Medicine, Tokyo, 160-8582, Japan.
Spinal Cord Ser Cases. 2021 Nov 5;7(1):97. doi: 10.1038/s41394-021-00461-y.
Gliofibroma is a rare tumor that develops in the brain and spinal cord. Due to the rarity of its nature, its pathophysiology and appropriate treatment remain elusive. We report a case of intramedullary spinal cord gliofibroma that was surgically treated multiple times. This report is of great significance because this is the first case of recurrence of this tumor.
A 32-year-old woman complained of gait disturbance and was referred to our institution. At the age of 13 years, she was diagnosed with intramedullary gliofibroma and underwent gross total resection (GTR) in another hospital. Based on imaging findings, tumor recurrence was suspected at the level of cervical spinal cord, and surgery was performed. However, the resection volume was limited to 50% because the boundary between the tumor and spinal cord tissue was unclear and intraoperative neuromonitoring alerted paralysis. At 1 year postoperatively, the second surgery was performed to try to resect the residual tumor, but subtotal resection was achieved at most. At 2 years after the final surgery, no tumor recurrence was observed, and neurologic function was maintained to gait with cane.
Although complete resection is desirable for this rare tumor at the initial surgery, there is a possibility to recur even after GTR with long-term follow-up. During surgical treatment for tumor recurrence, fair adhesion to the spinal cord is expected, and reoperation and/or adjuvant therapy might be considered in the future if the tumor regrows and triggers neurological deterioration.
神经胶质瘤纤维瘤是一种罕见的发生在脑和脊髓的肿瘤。由于其性质罕见,其病理生理学和适当的治疗方法仍然难以捉摸。我们报告了一例脊髓内神经胶质瘤纤维瘤多次手术治疗的病例。这一报告具有重要意义,因为这是该肿瘤复发的首例病例。
一名 32 岁女性因步态障碍就诊于我院。13 岁时,她被诊断为脊髓内神经胶质瘤纤维瘤,并在另一所医院接受了大体全切除(GTR)手术。根据影像学检查结果,怀疑颈段脊髓水平存在肿瘤复发,并进行了手术。然而,由于肿瘤与脊髓组织之间的边界不清楚,且术中神经监测提示有瘫痪风险,因此切除量仅为 50%。术后 1 年,进行了第二次手术以尝试切除残余肿瘤,但最多只能实现次全切除。最后一次手术后 2 年,未观察到肿瘤复发,且神经功能保持在借助拐杖行走的水平。
尽管在初次手术中,对于这种罕见肿瘤完全切除是理想的,但即使在 GTR 后也有可能复发,需要长期随访。在肿瘤复发的手术治疗过程中,预计会与脊髓有一定粘连,因此如果肿瘤再次生长并导致神经功能恶化,可能需要再次手术和/或辅助治疗。