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肿瘤性脊髓病

Neoplastic Myelopathies.

作者信息

Wu Jing, Ranjan Surabhi

出版信息

Continuum (Minneap Minn). 2018 Apr;24(2, Spinal Cord Disorders):474-496. doi: 10.1212/CON.0000000000000585.

Abstract

PURPOSE OF REVIEW

This article discusses the diagnosis and management of neoplasms that affect the spinal cord as well as spinal cord disorders that can occur due to cancer treatments.

RECENT FINDINGS

Neoplastic myelopathies are uncommon neurologic disorders but cause significant morbidity when they occur. Primary spinal cord tumors can be classified into intramedullary, intradural extramedullary, or extradural tumors. Diffuse gliomas and ependymal tumors are the most common intramedullary tumors. Diffuse gliomas include the World Health Organization (WHO) grade II and grade III astrocytomas, the grade II and grade III oligodendrogliomas, the grade IV glioblastomas, and newly recognized pediatric diffuse midline gliomas with H3 K27M mutation. The majority of diffuse and anaplastic astrocytomas are IDH-mutant tumors, whereas only 10% of glioblastomas are IDH-mutant. Oligodendrogliomas are typically IDH-mutant and are characterized by the molecular signature of 1p/19q codeletion. Nine distinct molecular subgroups of ependymomas have been identified based on their genetic features and location. NF2 mutations are frequently found in spinal cord ependymomas. Metastatic tumors are the most common tumors of the spine and can be extradural, leptomeningeal, or, rarely, intramedullary. Extradural metastatic spinal cord compression is a neurologic emergency and should be promptly diagnosed as pretreatment neurologic status dictates the posttreatment outcome.

SUMMARY

Neoplastic myelopathies encompass many diagnoses ranging from benign and malignant spinal tumors to paraneoplastic syndromes heralding cancers. The knowledge of the clinical features and management of neoplastic myelopathies is essential to practicing neurologists as early diagnosis and treatment can prevent devastating neurologic sequelae.

摘要

综述目的

本文讨论影响脊髓的肿瘤的诊断与管理,以及因癌症治疗可能发生的脊髓疾病。

最新发现

肿瘤性脊髓病是罕见的神经系统疾病,但一旦发生会导致严重的发病率。原发性脊髓肿瘤可分为髓内、髓外硬膜下或硬膜外肿瘤。弥漫性胶质瘤和室管膜瘤是最常见的髓内肿瘤。弥漫性胶质瘤包括世界卫生组织(WHO)二级和三级星形细胞瘤、二级和三级少突胶质细胞瘤、四级胶质母细胞瘤,以及新认识的具有H3 K27M突变的儿童弥漫性中线胶质瘤。大多数弥漫性和间变性星形细胞瘤是异柠檬酸脱氢酶(IDH)突变型肿瘤,而只有10%的胶质母细胞瘤是IDH突变型。少突胶质细胞瘤通常是IDH突变型,其特征为1p/19q共缺失的分子特征。根据室管膜瘤的基因特征和位置已确定了九个不同的分子亚组。神经纤维瘤病2型(NF2)突变在脊髓室管膜瘤中经常发现。转移性肿瘤是脊柱最常见的肿瘤,可位于硬膜外、软脑膜,或很少见的髓内。硬膜外转移性脊髓压迫是一种神经系统急症,应迅速诊断,因为治疗前的神经状态决定治疗后的结果。

总结

肿瘤性脊髓病涵盖从良性和恶性脊柱肿瘤到预示癌症的副肿瘤综合征等多种诊断。了解肿瘤性脊髓病的临床特征和管理对神经科医生至关重要,因为早期诊断和治疗可预防严重的神经后遗症。

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