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Myxopapillary Ependymomas

  • Ibrahim Omerhodžić
  • Mirza Pojskić
  • Kenan I. Arnautović
Chapter

Abstract

Myxopapillary ependymomas (MPEs) are World Health Organization (WHO) Grade I tumors that arise from the ependyma of the filum terminale located in the area of the conus medullaris and cauda equina. MPE is a slow-growing tumor most frequently found in adults between 30 and 50 years of age. They constitute around 13% of all ependymomas and as many as 90% of all tumors in the conus medullaris. The recommended treatment for patients with MPE is gross total resection; patients undergoing subtotal resection usually also undergo radiotherapy. Both microsurgical technique and spinal cord monitoring are indispensable to achieve total removal of MPEs and to obtain improvement of neurological recovery. Complete resection without capsular violation—the so called marginal en bloc resection—can be curative, and is often simply accomplished by resecting the filum above and below the mass. Nevertheless, this technique can be technically difficult in a given tumor based on tumor size, shape, and anatomical relation to the cauda equine nerves or spinal cord. Surgical morbidity in experienced hands is low with good chances for postoperative clinical improvements. Ten-year survival is reported to be 92–100% with 10-year progression-free survival (PFS) of 61.2%. Although myxopapillary ependymomas are classified as benign tumors, dissemination and local recurrence pose a major challenge in their clinical treatment. In the literature, dissemination and distant treatment failure are consistently described as occurring approximately 30% of the time. Recurrence rates have been reported around 15% following gross-total resection, and 30% following subtotal resection. Up to one-third of adult patients can display metastases at the initial diagnosis, which is why total spine magnetic resonance imaging (MRI) with MRI of the head is recommended in the initial workup. Radiotherapy may be recommended when a piecemeal resection is performed due to the rupture of the tumor capsule during surgery to prevent the local recurrence and CSF dissemination.

Keywords

Myxopapillary Ependymoma Gross Total resection Surgical technique Adjuvant radiotherapy Recurrence Primary seeding Secondary seeding 

Supplementary material

Video 15.1

Microsurgical resection of myxopapillary ependymoma of the lumbar spine in patient from Fig. 15.4. (MPG 432032 kb)

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Ibrahim Omerhodžić
    • 1
  • Mirza Pojskić
    • 2
  • Kenan I. Arnautović
    • 3
    • 1
  1. 1.Department of NeurosurgeryClinical Center University of SarajevoSarajevoBosnia and Herzegovina
  2. 2.Department of NeurosurgeryUniversity of MarburgMarburgGermany
  3. 3.Semmes MurpheyMemphisUSA

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