title: Multifocal intradural extramedullary ependymoma. Case report.
abstract: 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.
authors.affiliation: Departments of Neurology and NeurosurgeryUniversidade Federal de São Paulo, Brazil. eaiunes@ig.com.br
authors.forename: Eduardo Augusto
authors.initials: EA
authors.lastname: Iunes
authors.forename: João Norberto
authors.initials: JN
authors.lastname: Stávale
authors.forename: Rita
authors.initials: R
authors.lastname: de Cássia Caldas Pessoa
authors.forename: Ricardo
authors.initials: R
authors.lastname: Ansai
authors.forename: Franz Jooji
authors.initials: FJ
authors.lastname: Onishi
authors.forename: Manoel Antonio
authors.initials: MA
authors.lastname: de Paiva Neto
authors.forename: Antônio
authors.initials: A
authors.lastname: de Pádua Bonatelli
authors.forename: Sérgio
authors.initials: S
authors.lastname: Cavalheiro
authors.forename: Suzana M
authors.initials: SM
authors.lastname: Fleury Malheiros
mesh_terms: D000328:Adult; D001932:Brain Neoplasms / Q000175:diagnosis / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D003131:Combined Modality Therapy; D019299:Decompression, Surgical; D004806:Ependymoma / Q000175:diagnosis / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans; D007796:Laminectomy; D008279:Magnetic Resonance Imaging; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D009460:Neurologic Examination; D011183:Postoperative Complications / Q000175:diagnosis / Q000601:surgery; D018714:Radiotherapy, Adjuvant; D013117:Spinal Cord Compression / Q000175:diagnosis / Q000601:surgery; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*