The Results of Surgery, With or Without Radiotherapy, for Primary Spinal Myxopapillary Ependymoma: A Retrospective Study From the Rare Cancer Network

Pica, Alessia; Miller, Robert C.; Villà, Salvador; Kadish, Sidney P.; Anacak, Yavuz; Abusaris, Huda; Özyiğit, Gökhan; Baumert, Brigitta G.; Zaucha, Renata; Haller, Guy; Weber, Damien Charles

doi:10.1016/j.ijrobp.2008.09.034

Cited by 109 publications

(102 citation statements)

References 27 publications

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“…Se ha encontrado relación significativa con edad del paciente 1, , estado clínico 26 , afectación de esfínteres 38 , duración de los síntomas 6,11,26 , localización 26 , características de imagen con RM 34 , presencia o no de cápsula tumoral 34 , grado de resección tumoral 23,34 , tratamiento inicial y dosis de radioterapia 1 . Únicamente, Akyurek et al 1 en una serie de 35 pacientes encuentran como factores significativos de buen pronóstico la edad de los pacientes (<35 contra >35 años) y la modalidad de tratamiento inicial (cirugía contra cirugía y radioterapia).…”

Section: Discussionunclassified

Ependimomas del filum terminal. Análisis de 20 casos consecutivos

et al. 2010

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ResumenObjetivos. Analizar las características clínicas, radiológicas y los resultados quirúrgicos de una serie de ependimomas del filum terminal.Pacientes y método. Se estudia retrospectivamente 20 pacientes con 21 ependimomas del filum terminal tratados durante un período de 21 años . Todos los pacientes fueron diagnosticados con resonancia magnética e intervenidos quirúrgicamente.Resultados. La relación varón-mujer fue de 1:1.5 y la media de edad al diagnóstico de 44.8 años (rango 15-64). El primer síntoma fue dolor radicular (12 casos) y lumbalgia en los 8 restantes, con una duración media de la sintomatología antes del diagnostico de 8.7 años (rango 0.6-32). Todos los pacientes fueron intervenidos quirúrgicamente realizándose resección completa de 17 tumores y subtotal de 4. Histológicamente 20 tumores fueron ependimomas mixopapilares (grado I ) y un caso grado II. El período de seguimiento fue de 8 años (rango 1-18).Conclusiones. Los ependimomas del filum terminal, son tumores de crecimiento lento con una mayor incidencia en adultos jóvenes. La forma de presentación más habitual es con dolor lumbar con un largo tiempo de evolución. Aunque la mayoría son tumores de bajo grado histológico, tienen una especial tendencia a crecer y las recidivas locales no son raras.PALABRAS CLAVE: Ependimoma. Filum terminal. Resonancia magnética. Tumor medular. Tumor mixopapilar. Conclusions. Filum terminale ependimomas are slow growing tumours of the cauda equina with a high incidence in young adults. The most common presentation is with low back pain long time evolution. Although ependymomas of the filum terminale are thought to be benign, local recurrence is not uncommon.KEY WORDS: Ependymoma. Filum terminale. Magnetic resonance imaging. Myxopapillary tumor. Spinal cord tumor, IntroducciónLos ependimomas medulares son tumores poco frecuentes que constituyen menos del 5% de todos los tumores del sistema nervioso central (SNC) y entre el 10-15% de todos los tumores espinales 14,19 . Los ependimomas de la región lumbo-sacra representan el 30% de todos los ependimomas espinales y se trata habitualmente de ependimomas mixopapilares, variedad descrita por Kernohan en 1932 16 y que de acuerdo con la clasificación de la organización mundial de la salud (OMS) son tumores grado I. Clínicamente se caracterizan por presentarse con dolor lumbar con o sin irradiación ciática, por lo que se confun-

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Section: Discussionunclassified

Ependimomas del filum terminal. Análisis de 20 casos consecutivos

et al. 2010

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“…Other studies have generated more equivocal results. Pica and colleagues 27 and Schild and colleagues 33 found that adjuvant radiotherapy was associated with improved outcomes when given in high doses (> 50.4 Gy) but not when given in low doses. Agbahiwe and colleagues 1 showed that radiotherapy was associated with local tumor control in at least one subpopulation: pediatric patients.…”

Section: ©Aans 2013mentioning

confidence: 99%

“…The authors of several recent case series have examined the effect of the extent of resection on, and the role of adjuvant radiotherapy in, the treatment of MPEs. The majority of studies have been limited by small sample sizes: The largest published series to date included 85 patients with MPE, 27 and only 3 others included more than 40. 7,19,35 In the modern era, 2 main areas of controversy in the treatment of MPEs have emerged.…”

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confidence: 99%

Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children

Feldman

Clark²,

Safaee³

et al. 2013

SPI

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Object Myxopapillary ependymomas (MPEs) are rare WHO Grade I tumors found in the conus medullaris, cauda equina, and filum terminale. Treatment generally consists of resection with or without adjuvant radiotherapy. Evidence-based guidelines for surgical management are lacking due to the rarity of this tumor. Methods An English-language PubMed search was performed using the key words “myxopapillary” and “ependymoma.” Reports describing fewer than 3 patients or those lacking data on the extent of resection or radiotherapy were excluded. A total of 28 articles describing 475 patients met the authors' inclusion criteria. Patients were grouped by extent of resection and whether or not they underwent adjuvant radiotherapy. Differences in recurrence rates were assessed by chi-square test. Results The overall recurrence rate was 15.5% in patients treated by gross-total resection (GTR) and 32.6% in patients treated by subtotal resection (STR), irrespective of whether they underwent adjuvant therapy (p < 0.001). Regardless of the extent of resection, adjuvant radiotherapy was not associated with a decrease in recurrence rates. The overall recurrence rate was 15.6% in patients who underwent GTR and radiotherapy compared with 15.9% in patients who underwent GTR alone (p = 0.58), and it was 29.3% in patients who underwent STR and radiotherapy compared with 35.1% in those who underwent STR alone (p = 0.53). The difference between recurrence rates for patients who underwent GTR alone versus STR and radiotherapy was statistically significant (p = 0.02). Subgroup analysis demonstrated significantly higher recurrence rates in pediatric patients compared with adults (40.5% vs 23.4%, respectively; p = 0.02). Even in the setting of GTR alone, recurrence rates were higher in pediatric patients (65% vs 7.6%; p < 0.001). Conclusions Gross-total resection alone is associated with decreased recurrence rates compared with STR with or without radiotherapy. The authors' results suggest that treatment goals should include attempted GTR whenever possible. The observation that children benefitted from radiation therapy to a greater extent than did adults suggests that biological differences between tumors in these patient populations warrants more rigorous scientific studies.

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“…Although there is evidence that Grade I ependymomas are more aggressive in children compared with adults, 5,38,45 our institutional experience suggests that GTR can be achieved safely, in Table 1). Sagittal pre-(A) and postoperative (B) T1-weighted MR images after contrast administration from a patient with a spinal Grade II (classic) ependymoma.…”

Section: Discussionmentioning

confidence: 82%

“…38 Since Grade I ependymoma recurrences tend to be local, they can generally be treated by resection with or without radiotherapy. Radiotherapy may prove to be a useful adjunct and allow for less aggressive surgery with lower morbidity among patients with Grade I ependymomas.…”

Section: 238mentioning

confidence: 99%

Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults

Safaee¹,

Mummaneni

et al. 2014

PED

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Object Ependymomas are a common type of CNS tumor in children, although only 13% originate from the spinal cord. Aside from location and extent of resection, the factors that affect outcome are not well understood. Methods The authors performed a search of an institutional neuropathology database to identify all patients with spinal cord ependymomas treated over the past 20 years. Data on patient age, sex, clinical presentation, symptom duration, tumor location, extent of resection, use of radiation therapy, surgical complications, presence of tumor recurrence, duration of follow-up, and residual symptoms were collected. Pediatric patients were defined as those 21 years of age or younger at diagnosis. The extent of resection was defined by the findings of the postoperative MR images. Results A total of 24 pediatric patients with spinal cord ependymomas were identified with the following pathological subtypes: 14 classic (Grade II), 8 myxopapillary (Grade I), and 2 anaplastic (Grade III) ependymomas. Both anaplastic ependymomas originated in the intracranial compartment and spread to the spinal cord at recurrence. The mean follow-up duration for patients with classic and myxopapillary ependymomas was 63 and 45 months, respectively. Seven patients with classic ependymomas underwent gross-total resection (GTR), while 4 received subtotal resection (STR), 2 received STR as well as radiation therapy, and 1 received radiation therapy alone. All but 1 patient with myxopapillary ependymomas underwent GTR. Three recurrences were identified in the Grade II group at 45, 48, and 228 months. A single recurrence was identified in the Grade I group at 71 months. The mean progression-free survival (PFS) was 58 months in the Grade II group and 45 months in the Grade I group. Conclusions Extent of resection is an important prognostic factor in all pediatric spinal cord ependymomas, particularly Grade II ependymomas. These data suggest that achieving GTR is more difficult in the upper spinal cord, making tumor location another important factor. Although classified as Grade I lesions, myxopapillary ependymomas had similar outcomes when compared with classic (Grade II) ependymomas, particularly with respect to PFS. Long-term complications or new neurological deficits were rare. Among patients with long-term follow-up, those who underwent GTR had a recurrence rate of 20% compared with 40% among those with STR or biopsy only, suggesting that extent of resection is perhaps a more important prognostic factor than histological grade in predicting PFS, which has been suggested by other data in the literature. Given the relative paucity of these lesions, collaborative multiinstitutional studies are needed, and such efforts should also focus on molecular and genetic analysis to refine the current classification system.

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The Results of Surgery, With or Without Radiotherapy, for Primary Spinal Myxopapillary Ependymoma: A Retrospective Study From the Rare Cancer Network

Cited by 109 publications

References 27 publications

Ependimomas del filum terminal. Análisis de 20 casos consecutivos

Ependimomas del filum terminal. Análisis de 20 casos consecutivos

Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children

Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults

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