结节性硬化症中的脊柱巨细胞瘤：病例报告及文献复习
Spinal giant cell tumor in tuberous sclerosis: case report and review of the literature.
作者信息
Fraioli Mario Francesco, Lecce Mario, Fraioli Chiara, Paolo Curatolo
机构信息
University of Rome Tor Vergata, Rome, Italy.
出版信息
J Spinal Cord Med. 2013 Mar;36(2):157-60. doi: 10.1179/2045772312Y.0000000048.
BACKGROUND
Patients affected by tuberous sclerosis (TS) have a greater incidence of tumors than the healthy population. Spinal tumours in TS are reported very rarely and consist mainly of sacrococcygeal and cervical chordomas.
METHOD
Case report.
FINDINGS
A 21-year-old man, affected by TS, presented a spinal dorsal T2 tumor that caused medullary compression. He underwent decompressive laminectomy and microsurgical excision of a giant cell tumor and an associated aneurysmal bone cyst. Postoperative hypofractionated radiotherapy was performed on the surgical field. At 2.4 years of follow-up the patient reported total recovery of neurological deficits and was free from tumor recurrence.
CONCLUSION
Considering this association, which is the first reported in the literature, spinal magnetic resonance imaging with gadolinium should be performed at the onset of spinal pain in patients affected by TS.
背景
结节性硬化症（TS）患者的肿瘤发病率高于健康人群。TS患者的脊柱肿瘤报道非常罕见，主要为骶尾部和颈椎脊索瘤。
方法
病例报告。
结果
一名21岁的TS患者出现脊髓背侧T2肿瘤，导致脊髓受压。他接受了减压椎板切除术，并对一个巨细胞瘤和一个相关的动脉瘤样骨囊肿进行了显微手术切除。术后对手术区域进行了低分割放疗。随访2.4年时，患者报告神经功能缺损完全恢复，且无肿瘤复发。
结论
考虑到这种关联是文献中首次报道的，对于TS患者，在出现脊柱疼痛时应进行钆增强脊柱磁共振成像检查。
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