Multicentric extramedullary myxopapillary ependymomas: Two case reports and literature review

Landriel, Federico; Ajler, Pablo; Tedesco, Nicolas; Bendersky, Damián; Vecchi, Eduardo

doi:10.4103/2152-7806.100859

Cited by 19 publications

(28 citation statements)

References 15 publications

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“…With the exception of 2 unoperated patients presenting with large ependymomas, all patients demonstrating subarachnoid dissemination had undergone incomplete tumor removal, suggesting that dissemination is an iatrogenic phenomenon in the majority of instances. 28 Dissemination is unusual in previously unoperated patients 16,18,24,36 and was restricted to large tumors without capsules in this series. Therefore, the entire spinal axis should be examined on MRI whenever a large tumor extending over more than 4 spinal segments or a recurrent tumor is encountered.…”

Section: Discussion Clinical Presentation and Diagnosismentioning

confidence: 97%

Spinal ependymomas. Part 2: Ependymomas of the filum terminale

Klekamp

2015

FOC

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OBJECT Ependymomas of the filum terminale provide specific surgical challenges due to their often enormous size, contact with nerve roots of the cauda equina and conus, and potential for subarachnoid dissemination. This study presents treatment results for these tumors over a 30-year period. METHODS Among 1447 patients with tumors of the spinal canal treated between 1980 and 2014, 618 patients presented with extramedullary tumors. Of these, 42 patients (25 males, 17 females) demonstrated a myxopapillary ependymoma in the lumbosacral region. Thirty-four patients underwent 36 operations for 39 such tumors. The mean patient age was 38 ± 14 years (range 11−73 years), with an average clinical history of 37 ± 67 months. Patients were followed through outpatient visits and questionnaires, with a mean follow-up of 10 years (127 ± 100 months). Twenty-seven operations were performed to treat de novo tumors and the remainder were undertaken on recurrent tumors. Short-term results were determined for individual symptoms, and tumor recurrence rates were calculated with Kaplan-Meier statistical analyses. RESULTS Subarachnoid dissemination was observed in 11 patients and was related to previous surgery in 9 patients and associated with extensive tumors in 2 patients. Gross-total resections (GTR) were achieved in 28 operations (77.7%) and subtotal resections in the remainder. Subtotal resections were restricted to unencapsulated ependymomas (61.5%). Radiotherapy was employed after 6 operations on unencapsulated tumors, with 5 of these also demonstrating subarachnoid seeding. Permanent surgical morbidity affected 3 patients who experienced permanent worsening of bladder function, while 7 patients showed no postoperative changes, and the remaining 26 operations were followed by improvements. Long-term outcome depended on the amount of resection and the presence of a tumor capsule. Eight of 9 tumor recurrences affected unencapsulated tumors, of which 3 had undergone GTR. The overall recurrence rates were 6.6%, 19.0%, and 37.0% after 1, 10, and 20 years, respectively. For unencapsulated ependymomas, the corresponding rates were 15.6%, 32.5%, and 66.2% after 1, 10, and 20 years, respectively, with significantly lower rates of 9.1% after 10 and 20 years for encapsulated tumors. Postoperative radiotherapy tended to prolong the recurrence-free interval for patients with unencapsulated tumors. Five patient deaths occurred during follow-up, of which 2 deaths were tumor related and occurred at 216 and 287 months after surgery. CONCLUSION Extramedullary ependymomas are slow-growing tumors in the lumbosacral region, sometimes with an indolent course for long periods of time. Despite their delicate location and often enormous size, surgical morbidity in experienced hands is low, with good chances for postoperative clinical improvements and very low recurrence rates after GTR for encapsulated tumors. The role of postoperative radiotherapy remains controversial. Radiotherapy may be considered after incomplete resections of unencapsulated tumors and/or for patients with subarachnoid dissemination.

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Section: Discussion Clinical Presentation and Diagnosismentioning

confidence: 97%

Spinal ependymomas. Part 2: Ependymomas of the filum terminale

Klekamp

2015

FOC

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“…[ 1 ] Though 20 cases of purely IDEM ependymomas have been reported in the literature, in most of these cases, single lesion was found. [ 1 2 3 4 ] Our case had multiple lesions scattered throughout the spinal axis. Only 4 such cases have been described in the literature [ Table 1 ].…”

Section: Discussionmentioning

confidence: 81%

“…[ 1 ] Intradural extramedullary (IDEM) ependymomas subtype are extremely rare. [ 1 2 3 4 ] Multiple IDEM ependymomas are even rarer. Only 4 such cases have been reported until.…”

Section: Introductionmentioning

confidence: 99%

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Multicentric intradural extramedullary ependymoma: Report of a rare case

Vats

Ramdasi

Zaveri

et al. 2015

J Craniovert Jun Spine

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Spinal ependymoma commonly presents as an intramedullary tumor. We present a rare case of multicentric intradural extramedullary spinal ependymoma. A 59 years old female presented to us with spastic quadriparesis for 10 months. Magnetic resonance imaging of the spinal cord showed discretely located enhancing tumor masses from at C1-C2, C6-C7, and D4 to L3 level. Subtotal resection of the symptomatic tumor at C6-C7 and D7-D9 was done. The patient underwent radiotherapy with 50.4 Gy. At follow-up of 11 months, patient is doing well. The relevant literature is reviewed.

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“…[11] Las lesiones intradurales con resección completa tienen una sobrevida media de 19 años, en cambio las lesiones extradurales tienen mal pronóstico con un alto porcentaje de recidivas y metástasis. [15]…”

Section: Discussionunclassified

Ependimoma myxopapilar sacro gigante con osteolisis Giant osteolitic sacral myxopapillary ependymoma

Ajler¹,

Landriel²,

Goldschmidt³

et al. 2014

Surg Neurol Int

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Objetivo:la presentación de un caso de una paciente con un ependimoma sacro con extensa infiltración y destrucción ósea local.Descripción del caso:una mujer de 53 años acudió a la consulta por dolor lumbosacro y alteraciones sensitivas perineales y esfinterianas. La imágenes por Resonancia Magnética (IRM) y la Tomografía Axial Computada (TAC) mostraron una lesión expansiva gigante a nivel S2-S4 con extensa osteólisis e invasión de tejidos adyacentes. Se realizó una exéresis tumoral completa con mejoría del estatus funcional. La anatomía patológica informó ependimoma mixopapilar.Discusión:la extensión de la resección quirúrgica es el mejor predictor de buen pronóstico. El tratamiento radiante se reserva como opción adyuvante para las resecciones incompletas y recidiva tumoral. La quimioterapia sólo debería utilizarse en casos en que la cirugía y la radioterapia estén contraindicadas.Conclusión:Los ependimomas mixopapilares sacros con destrucción ósea y presentación intra y extradural son muy infrecuentes y deben ser tenidos en cuenta entre los diagnósticos diferenciales preoperatorios. Su resección total, siempre que sea posible, es la mejor alternativa terapéutica.

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Multicentric extramedullary myxopapillary ependymomas: Two case reports and literature review

Cited by 19 publications

References 15 publications

Spinal ependymomas. Part 2: Ependymomas of the filum terminale

Spinal ependymomas. Part 2: Ependymomas of the filum terminale

Multicentric intradural extramedullary ependymoma: Report of a rare case

Ependimoma myxopapilar sacro gigante con osteolisis Giant osteolitic sacral myxopapillary ependymoma

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