Surgery for Spinal Cord Ependymomas: Outcome and Prognostic Factors

Boström, Azize; Lehe, Marec von; Hartmann, Wolfgang; Pietsch, Torsten; Feuss, Mareike; Boström, Jan; Schramm, Johannes; Simon, Matthias

doi:10.1227/neu.0b013e3182004c1e

Cited by 132 publications

(67 citation statements)

References 18 publications

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“…Gross total resection has been shown to reduce the risk for recurrence and increase progression-free survival (PFS) [5, 7, 30]. In cases with involvement of the conus, the likelihood of complete resection is reduced or implies an increased risk of postoperative morbidity [2].…”

Section: Discussionmentioning

confidence: 99%

Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients

et al. 2014

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MethodsTen consecutive children (0–18 years) who underwent primary tumor resection for a spinal ependymoma between 1980 and 2011 were included in this retrospective study. Gross motor function and activities of daily life were scored according to the Barthel Index.ConclusionThree out of six pediatric patients treated for spinal myxopapillary ependymoma are disease-free after 11 to 33 years of follow-up. The other three have progressive disease, after relapses occurring after 4.5, 7, and 20 years, respectively. One out of two patients with grade II ependymoma had progressive disease from 10 years after initial surgery but is in full-time work in spite of widespread metastatic disease after 32 years. One of the two children with grade III tumor died from progressive disease 17 years from primary diagnosis, while the last one is tumor-free after 19 years. The quality of life is good for three of the four patients with widespread disease, and they are managed conservatively aiming at symptomatic treatment intervention if necessary. We strongly advocate lifelong follow-up for children treated for spinal ependymomas.

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Section: Discussionmentioning

confidence: 99%

Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients

et al. 2014

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“…The tumor bed is the main site of failure [3], and metastasis is uncommon [35]. Long-term development of secondary neoplasms has been observed in this patient population [8, 36, 37]. …”

Section: Introductionmentioning

confidence: 99%

Prognosis by tumor location in adults with intracranial ependymomas

Sayegh

Aranda

Kim

et al. 2014

Journal of Clinical Neuroscience

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Intracranial ependymomas are rare tumors in adults. Thus, factors affecting prognosis are poorly understood. We performed a study to investigate whether tumor location is an important prognostic factor in adults who undergo surgery for intracranial ependymomas. PubMed was searched to identify studies that reported clinical outcomes in adult patients with intracranial ependymoma. Data were extracted for patient and tumor characteristics, extent of resection, progression-free survival (PFS), and overall survival (OS). Tumors were categorized as supratentorial or infratentorial and extraventricular or intraventricular. Presenting clinical features and tumor characteristics were tabulated. Kaplan–Meier and multivariate Cox regression survival analyses were performed to determine PFS and OS by tumor location. Extent of resection was also analyzed by tumor location. A total of 183 patients were included in the metaanalysis. Patients presented at a mean of 8.2 months with a myriad of clinical features. The mean tumor size was 3.38 cm, and 19.3% of tumors were cystic. Supratentorial tumors were most commonly located in the frontal and parietal lobes, and infratentorial tumors in the fourth ventricle. Supratentorial tumors demonstrated significantly poorer PFS (p < 0.001) and OS (p = 0.003) than infratentorial tumors, despite a higher rate of gross total resection (GTR) for the supratentorial tumors (72.6% versus 42.1%). Extraventricular ependymomas displayed significantly poorer PFS than intraventricular ependymomas (p = 0.009). In summary, supratentorial ependymomas have significantly poorer PFS and OS than their infratentorial counterparts, despite being more conducive to GTR, suggesting increased clinical aggressiveness. Extraventricular location is also associated with significantly poorer PFS than intraventricular location.

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“…Multiple previous reports have investigated outcomes based on extent of surgical resection. It is a near universal finding that GTR improves outcomes, often measured as progression-free survival (PFS); while not statistically significant in this cohort there was a trend towards improvement in OS in patients receiving GTR in this analysis [6][7][8][9][10][11][12][13][14]. The few mean tumor size among those receiving RT (4.6 cm) was significantly larger than among those not receiving RT (3.2 cm, p = 0.0002).…”

Section: Discussionmentioning

confidence: 54%

Myxopapillary ependymoma: a SEER analysis of epidemiology and outcomes

2016

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Myxopapillary ependymoma (MPE) is an exceedingly rare tumor histology. While surgery is clearly the treatment of choice, controversy exists regarding the role of adjuvant radiotherapy (RT). Using the Surveillence, epidemiology, and end results (SEER) database, we aimed to determine the epidemiology, prognostic factors, and treatment-related outcomes for MPE. A total of 773 cases were found in the SEER database. The incidence in the American population was found to be 1.00 per million person-years. On multivariate analysis, receipt of surgery (HR = 0.14, CI = 0.06-0.35, p < 0.001), receipt of RT (HR = 4.06, CI = 1.87-8.81, p < 0.001), age less than 30 (HR = 0.24, CI = 0.08-0.72, p = 0.01), and Caucasian race (HR = 0.37, CI = 0.13-0.996, p = 0.049) were statistically significant prognostic factors. The mean tumor size among those receiving RT (4.6 cm) was significantly larger than among those not receiving RT (3.2 cm, p = 0.0002). Those who lived in metropolitan areas were more likely to receive RT than those who did not. Given multiple previous studies show that RT improves PFS and the discrepancy in tumor size, selection bias is likely a significant contributor to the apparent negative impact of RT on OS. Regardless, surgery remains the most crucial aspect in the care of patients with MPE.

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Surgery for Spinal Cord Ependymomas: Outcome and Prognostic Factors

Abstract: These results support early surgery aiming at complete resection as the primary treatment for presumed spinal ependymomas. The prognosis after surgery for some myxopapillary ependymomas seems worse than generally believed.

Cited by 132 publications

References 18 publications

Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients

Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients

Prognosis by tumor location in adults with intracranial ependymomas

Myxopapillary ependymoma: a SEER analysis of epidemiology and outcomes

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