Neoplastic Myelopathies.

Abstract

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. 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. 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.