免疫功能低下个体中的多灶性脊柱恶性周围神经鞘瘤：病例报告及文献复习
Multifocal spinal malignant peripheral nerve sheath tumor in an immunocompromised individual: case report and review of literature.
作者信息
Roopesh Kumar V R, Madhugiri Venkatesh S, Sasidharan Gopalakrishnan M, Shankar Ganesh C V, Gundamaneni Sudheer Kumar
机构信息
Department of Neurosurgery, Jawaharlal Institute of Post-graduate Medical Education and Research (JIPMER), 2nd Floor, Super Specialty Block, Pondicherry, 605006, India,
出版信息
Eur Spine J. 2014 May;23 Suppl 2:236-41. doi: 10.1007/s00586-013-3103-z. Epub 2013 Nov 20.
PURPOSE
Primary intraosseous spinal malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. MPNST with multifocal origin has been described to occur in the extremities. Such a lesion has not been described to occur in the spine. We describe a case of multifocal spinal MPNST and to review the literature relevant to this rare entity and its management.
METHODS
A 40-year-old immunodeficient patient presented with rapidly progressive paraparesis and mid back ache.
RESULTS
Despite aggressive surgical decompression, he developed multiple metastases 3 months after surgery. However, he remained stable for 1 year without any adjuvant therapy. Presently, he has received palliative radiotherapy for spinal recurrence and cerebral metastasis.
CONCLUSION
Multifocal spinal MPNST is a rare lesion. In this instance, the multifocality of the disease and its odd location could be attributed to the immunodeficiency state. The prolonged survival could be due to an improvement in his immune status due to HAART.
目的
原发性骨内脊柱恶性外周神经鞘瘤（MPNST）极为罕见。多灶性起源的MPNST已被描述发生于四肢。尚未见有此类病变发生于脊柱的报道。我们报告一例多灶性脊柱MPNST病例，并复习与此罕见实体及其治疗相关的文献。
方法
一名40岁的免疫缺陷患者出现快速进展的双下肢轻瘫和中背部疼痛。
结果
尽管进行了积极的手术减压，他在术后3个月出现了多处转移。然而，在未接受任何辅助治疗的情况下，他病情稳定了1年。目前，他因脊柱复发和脑转移接受了姑息性放疗。
结论
多灶性脊柱MPNST是一种罕见病变。在此病例中，疾病的多灶性及其特殊位置可能归因于免疫缺陷状态。生存期延长可能是由于高效抗逆转录病毒治疗（HAART）使他的免疫状态得到改善。
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