Multifocal intradural extramedullary ependymoma

Case report

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In this paper, the authors present the case of a patient with multifocal intradural extramedullary ependymoma, and they review 18 previously reported cases.

A 32-year-old man presented to the authors' institution with a 1-month history of partial medullary syndrome. Magnetic resonance imaging of the neuraxis revealed multifocal intradural extramedullary lesions at the bulbomedullary junction and C2–3, T5–11, L-2, L-4, L-5, and sacrum. Histological examination revealed a WHO Grade II ependymoma.

The literature survey yielded 18 cases of ependymoma at the same location; none of them were multifocal at presentation. The authors analyzed the epidemiological, clinical, and surgical features of all 19 cases reported to date, including the present case.

Patients' ages ranged from 24 to 69 years; 15 patients were women and 4 were men. The time elapsed from symptom onset to diagnosis ranged from 1 month to 8 years. Pain (in 13 patients) and medullary syndrome (in 12) were reported as the initial symptoms (information was not provided for 1 patient). Tumors were predominantly located in the thoracic spine (11), but they also occurred in the cervicothoracic (3), cervical (2), and lumbar (2) spine. The remaining tumor was multifocal. Solitary extramedullary tumors were found intraoperatively in 13 patients; 3 were described as exophytic and 3 as extramedullary with some degree of medullary invasion. Histological examination revealed 9 WHO Grade II tumors, 4 Grade III tumors, and 1 myxopapillary tumor. Data obtained for the remaining cases proved inconclusive. The clinical condition improved in 11 patients, remained stable in 2, and worsened (recurrence or progression) in 6. Of the 4 patients with Grade II tumors who presented with recurrence or neuraxis spreading, 3 had meningeal infiltration or adhesion to the pia mater, which does not rule out the possibility of neoplastic remnants in that area.

Intradural extramedullary ependymomas are rare, they predominate in women in the 5th decade of life, and pain is the most frequent initial symptom. The extent of resection and the presence of meningeal infiltration seem to be key determinants of prognosis. The present case is the first intradural extramedullary ependymoma (with the exception of those occurring at the conus medullaris and terminal filum) with multiple lesions at presentation.

Article Information

Address correspondence to: Eduardo Augusto Iunes, M.D., Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, Rua Napoleão de Barros, 715/6th floor, São Paulo, SP, Brazil, 04024002. email: eaiunes@ig.com.br.

Please include this information when citing this paper: published online December 10, 2010; DOI: 10.3171/2010.9.SPINE09963.

© AANS, except where prohibited by US copyright law.

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Figures

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    Sagittal T2-weighted (A and C) and fat-suppressed, postcontrast T1-weighted (B and D) MR images of the cervicothoracic region demonstrating multiple intradural extramedullary lesions in the bulbomedullary junction, C2–3, and T6–11 with high signal intensity on the T2-weighted images, cystic areas, and moderate and heterogeneous enhancement. Note the irregular and thickened enhancement outlining the posterior surface of spinal cord.

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    Sagittal fat-suppressed postcontrast T2-weighted (left) and T1-weighted (right) MR images of the lumbar spine demonstrating intradural and extramedullary entities appearing as cervicothoracic lesions at the level of L-4 and L-5. Superficial enhancement is observed in the conus medullaris.

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    Postcontrast FLAIR (left) and T1-weighted (right) MR images of the brain showing an interpeduncular nodular lesion hyperintense on the FLAIR image and with mild contrast enhancement.

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    Photomicrographs showing a moderately cellular ependymoma with a monomorphic nuclear morphology and a perivascular pseudorosette (A), perivascular pseudorosette (B), increased cellularity and pleomorphic area with 1 mitosis (C), and ependymal rosette (D). H & E, original magnification × 100 (A and B); × 400 (C and D).

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