Fleming Karen L, Davidson Laurence, Gonzalez-Gomez Ignacio, McComb J Gordon
Division of Neurosurgery, Childrens Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California 90027, USA.
J Neurosurg Pediatr. 2010 Feb;5(2):172-8. doi: 10.3171/2009.9.PEDS09238.
Intramedullary spinal cord lipomas not associated with dysraphism are infrequently reported. When present, they typically occur in children and have a predilection for the cervical and thoracic spinal cord. The authors review the presentation, treatment, and disease course in 5 pediatric patients, and compare the outcomes with previously reported cases.
With institutional review board approval, a retrospective chart review was undertaken at Children's Hospital Los Angeles.
Four patients with intramedullary spinal cord lipomas and 1 patient with a lipoblastoma, none associated with dysraphism, were retrospectively reviewed. There were 2 boys and 3 girls ranging in age from 2 months to 4 years. Four patients underwent a laminectomy or laminoplasty with one or more subtotal resections. One patient initially underwent a decompressive laminoplasty without debulking. The median follow-up was 8 years (range 12 months-11 years). Two patients had regrowth of their lipoma, necessitating a second surgery in one patient and 3 debulking surgeries in the other. Postoperatively, 3 patients developed mild kyphosis, none significant enough to require orthopedic intervention. One patient underwent a stabilization procedure at the time of the initial laminectomy and tumor debulking. No patient received chemotherapy or radiation. At the most recent follow-up visit, patients demonstrated improved neurological function when compared with preoperative status.
In addition to a decompressive laminectomy, debulking of the lipoma provides the best long-term neurological outcome. Gross-total excision is not warranted and usually is not possible. Long-term follow-up is needed, and repeat debulking of the lipoma is indicated if there is an increase in tumor size due to hyperplasia of residual adipocytes, when tumor growth is associated with neurological deterioration.
髓内脊髓脂肪瘤且不伴有脊柱裂的病例鲜有报道。若存在此类情况,通常发生于儿童,且好发于颈段和胸段脊髓。作者回顾了5例儿科患者的临床表现、治疗及病程,并将结果与既往报道的病例进行比较。
经机构审查委员会批准,在洛杉矶儿童医院进行了一项回顾性病历审查。
对4例髓内脊髓脂肪瘤患者和1例脂肪母细胞瘤患者进行了回顾性分析,所有病例均不伴有脊柱裂。患者年龄从2个月至4岁,其中2例为男孩,3例为女孩。4例患者接受了椎板切除术或椎板成形术,并进行了一次或多次次全切除。1例患者最初接受了减压性椎板成形术,但未进行肿瘤切除。中位随访时间为8年(范围12个月至11年)。2例患者脂肪瘤复发,其中1例需要再次手术,另1例需要进行3次肿瘤切除手术。术后,3例患者出现轻度脊柱后凸,但均未严重到需要骨科干预。1例患者在初次椎板切除术和肿瘤切除时接受了稳定手术。所有患者均未接受化疗或放疗。在最近一次随访时,与术前状态相比,患者的神经功能有所改善。
除减压性椎板切除外,脂肪瘤切除可提供最佳的长期神经功能结果。无需进行全切除,而且通常也不可能做到。需要进行长期随访,如果由于残留脂肪细胞增生导致肿瘤大小增加,或者肿瘤生长与神经功能恶化相关,则需要再次切除脂肪瘤。