Ependymoma in Adults: Surgery, Reoperation and Radiotherapy for Survival

Vitanovics, Dusan; Bálint, Katalin; Hanzély, Zoltán; Banczerowski, Péter; Afra, D

doi:10.1007/s12253-009-9194-5

Cited by 15 publications

(15 citation statements)

References 49 publications

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“…To date, radical resection is the widely accepted therapy for ependymoma (19). Adjuvant radiotherapy and chemotherapy have been used post-operatively to prevent tumor recurrence.…”

Section: Discussionmentioning

confidence: 99%

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Primary cerebellopontine angle ependymoma with spinal metastasis in an adult patient: A case report

et al. 2015

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Abstract. Subtentorial ependymoma is a common central nervous system tumor in young children, but is uncommon in adults. Ependymoma often arises from the cells lining the fourth ventricle. The present study reports a rare case of primary ependymoma that originated from the cerebellopontine angle, with local extension to the two internal auditory canals and remote spinal metastasis, in an adult male. A 50-year-old male presented with headache, tinnitus and bilateral hearing loss that had persisted for 4 months. Magnetic resonance imaging (MRI) revealed a mass in each of the cerebellopontine angles, which had spread to each internal auditory canal and wrapped the VII/VIII cranial nerve complex. A gross total resection was performed to remove the mass in the right side. Histological examination confirmed that the tumor was a World Health Organization grade II papillary ependymoma. Notably, the patient complained of urine retention post-surgery and massive occupational lesions in T3-T4 and L5-S2 were found on full spinal cord MRI. The patient then received combination therapy consisting of temozolomide, and whole-brain and spinal cord radiation. In the final follow-up examination, performed 13 months after treatment, slight shrinkage of the T3 lesion was observed, and no progression of the left cerebellopontine angle and S5-L2 lesions were identified on MRI. In summary, although this clinical entity is rare, the diagnosis of ependymoma and the possibility of spinal cord metastasis should be considered in subtentorial tumors. IntroductionSubtentorial ependymoma mainly occurs in young children and usually arises in the fourth ventricle (1). Although it is a relatively benign tumor, cerebrospinal fluid (CSF) spread is found in 8-33% of patients (2,3). Moreover, subtentorial ependymoma is more inclined to exhibit CSF metastasis compared with supratentorial ependymoma (4).Bilateral primary posterior fossa ependymoma originating from the cerebellopontine angle, termed primary cerebellopontine angle ependymoma, is a rare form of subtentorial ependymoma that predominantly occurs in infants and young children. The main symptom is headache due to progressively increasing intracranial pressure (5), while cranial nerve deficit is relatively uncommon. Surgical resection with subsequent radiotherapy is the primary treatment strategy for patients with ependymoma (6). The efficacy of conventional chemotherapy for this disease remains uncertain; however, temozolomide appears to be a promising adjuvant therapeutic approach for multifocal anaplastic ependymoma following surgical resection (7). The extent of surgical resection is the major determinant of overall survival in pediatric patients (8). However, the prognosis varies in different reports, with 5-year overall survival ranging between 50 and 64%, and progression-free survival ranging between 23 and 64% (5,9-11).The present study reports a rare case of primary cerebellopontine angle ependymoma extending to the internal auditory canals and spinal cord in an adult man. To the best...

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“…To date, radical resection is the widely accepted therapy for ependymoma (19). Adjuvant radiotherapy and chemotherapy have been used post-operatively to prevent tumor recurrence.…”

Section: Discussionmentioning

confidence: 99%

“…Chemotherapy has also been used to decrease the tumor size prior to surgery and reduce the radiotherapy dosage post-surgery. However, the potential for adjuvant radiotherapy to improve the prognosis of a patient with ependymoma remains under debate (19)(20)(21).…”

Section: Discussionmentioning

confidence: 99%

Primary cerebellopontine angle ependymoma with spinal metastasis in an adult patient: A case report

et al. 2015

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“…As the incidence of ependymoma in the general population is very low, there are considerable controversies over its therapeutic outcome and factors affecting the prognosis [1,9]. Several prognostic factors have been proposed, including age (pediatric vs. adult population), location (infratentorial vs. supratentorial), histology (grade 2 vs. grade 3), surgical resection extent (total vs. partial removal), and efficacy of radiotherapy [7][8][9][10][11][12][13]. However, the statistic value of these prognostic factors is limited because of the low incidence and small sample size.…”

Section: Discussionmentioning

confidence: 99%

“…In this study, surgical resection was performed in all patients with different approaches according to the location of the tumor under the surgical microscope similarly with the reports before [2,11,[15][16][17]. Surgical resection extent was determined according to the surgical reports and postoperative imaging study.…”

Section: Surgery and Adjuvant Therapymentioning

confidence: 99%

“…Although age, surgical resection extent and histology have emerged as the most significant prognostic predictors of intracranial ependymomas [9][10][11][12][13], whether different age, surgical resection extent or malignancy grade would affect the other factors, or even indirectly affect the prognosis remains unanswered. We therefore analyzed the correlations between the prognostic factors, and the results are shown in Table 2 and Supplemental Table 1.…”

Section: Correlations Between the Prognostic Factorsmentioning

confidence: 99%

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Analysis on therapeutic outcomes and prognostic factors of intracranial ependymoma: a report of 49 clinical cases in a single center

Zhu

Yan

et al. 2015

Neurol Sci

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Although intracranial ependymoma is relatively rare, it is often associated with great clinical aggressiveness and poor overall survival. There are controversies over factors affecting the prognosis of the disease. The aim of this retrospective study was to evaluate factors that may affect the therapeutic outcome and prognosis of intracranial ependymoma by reviewing the medical records of 49 patients who were surgically treated in our hospital between 2001 and 2014. Univariate and multivariate analyses were performed to identify prognostic variables relative to patient and tumor characteristics, and treatment modalities. All 49 patients (24 men and 25 women; mean age 27.6 years) underwent surgical resection, of whom 14 patients also underwent postoperative radiotherapy. Local recurrence was found in 15 (48.8 %) patients, and 22 (51.2 %) patients died during the follow-up periods. The 5-year recurrence rate was 65 % and the survival rate was 51 %. The results of statistical analysis suggested that preoperative extraventricular drainage and surgical resection extent were prognostic factors related to progression-free survival, and that age, surgical resection extent and histological grade were closely associated with survival. Interestingly, there was a significant correlation between the symptom of hydrocephalus and age (P = 0.010), and patients with a better clinical status (KPS ≥ 80) were significantly associated with a lower WHO grade (P = 0.007). In conclusion, we confirmed that surgical resection extent was the major independent factor affecting both recurrence and survival of patients with intracranial ependymoma, while age and WHO grade were prognostic factors affecting survival but not recurrence.

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Clinical course of adult patients with ependymoma

Armstrong

Vera-Bolaños

Gilbert

2011

Cancer

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BACKGROUND: Ependymomas are a rare tumor in adults, and there are limited reports of the clinical course, treatment, and current health status of patients. METHODS: Patients with ependymoma completed an online survey regarding their diagnosis, treatment course, and current health status. Descriptive statistics were used to report the characteristics and degree of symptom severity. Correlations between demographic and clinical characteristics were explored with univariate analysis. RESULTS: One hundred eighteen adults participated, and there were more women (n ¼ 68) than men (n ¼ 50). The median age was 48 years (range, 22-77 years). Fifty-nine participants (48%) reported that they had not been able to work since their diagnosis, and 31% reported receiving disability benefits. Patients who had been treated for spine tumors had symptoms longer than those who had been treated for brain lesions (chi-square statistic, 7.294; P ¼ .026), and the majority had 3 symptoms before diagnosis. Most patients reported undergoing complete resection (brain lesions, 62%; spine tumors, 58%), and patients with spine tumors were less likely to have received additional treatment (chi-square statistic, 9.687; P ¼ .008). The majority had not had a recurrence and reported consulting a neurosurgeon for surveillance. Despite having stable disease and not receiving active treatment, most patients described moderate to severe symptoms, including fatigue (44%), numbness/tingling (39%), pain (36%), and disturbed sleep (34%) overall. Brain lesions were associated with altered vision (25%), difficulty concentrating (25%), weakness (19%), irritability (19%), difficulty speaking (19%), and understanding (17%); and spine lesions were associated with extremity weakness (55%), sexual dysfunction (48%), radiating pain (37%), and change in bowel pattern (35%). CONCLUSIONS: Treatment for adult patients with ependymoma is not standardized. Despite the low recurrence rate, patients reported significant symptoms and disability. Cancer 2011;117:5133-

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Ependymoma in Adults: Surgery, Reoperation and Radiotherapy for Survival

Abstract: In our experience the use of radiotherapy in adult, intracranial WHO Grade II ependymoma patients had no significant effect on prognosis. Radical surgery and eventual reoperation seems to be more favorable.

Cited by 15 publications

References 49 publications

Primary cerebellopontine angle ependymoma with spinal metastasis in an adult patient: A case report

Primary cerebellopontine angle ependymoma with spinal metastasis in an adult patient: A case report

Analysis on therapeutic outcomes and prognostic factors of intracranial ependymoma: a report of 49 clinical cases in a single center

Clinical course of adult patients with ependymoma

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