• Kuntal Kanti Das, Gagandeep Attri, Suyash Singh, Kamlesh Singh Bhaisora, Pawan Verma, Sushila Jaiswal, Awadeshkumar Jaiswal, and Sanjay Behari.
• Department of Neurosurgery, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India. Electronic address: kkdas@sgpgi.ac.in.
• World Neurosurg. 2020 Feb 1; 134: 323-336.

BackgroundEpendymomas are common intramedullary spinal tumors but there are scattered reports of this tumor presenting with exophytic growth patterns masquerading as intradural extramedullary (IDEM) tumors. Such IDEM ependymomas are seldom suspected preoperatively and it is only during surgery that their existence is revealed. Little is known of such rare growth patterns of an otherwise common intramedullary spinal cord tumor, their characteristics, and their management considerations.MethodsWe present a case of an exophytic dorsal ependymoma with a stalk like attachment to the spinal cord and the surgical management in a 24-year old woman. An extensive literature search was carried out on all prominent databases to find out similar cases reported earlier. We excluded filum/conus ependymoma, purely extradural spinal ependymoma as well as the extraspinal ependymomas. Details of each case reported before were obtained and tabulated.ResultsIDEM ependymomas have been reported in 54 patients so far, including the present case. Four patterns of growth emerged from the literature review: intramedullary ependymoma with exophytic component (group I, n = 9), exophytic IDEM ependymoma without intramedullary component (group II, n = 6), IDEM ependymoma arising from nerve roots (group III, n = 7), and pure IDEM ependymoma (group IV, n = 32). Except in group I, IDEM ependymoma affects females more frequently, without any specific age predilection. Thoracic spinal cord/canal is the most common location across all groups. Multifocal disease, craniospinal dissemination, and recurrences tend to be maximum in group IV.ConclusionsIDEM ependymomas are more common in thoracic segment of the cord and broadly divisible into 4 subgroups. We suggest a subpial origin of group II IDEM ependymomas. We also advocate proliferation index estimation in grade II ependymomas to enable formulation of an optimal management plan.Copyright © 2019 Elsevier Inc. All rights reserved.

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