Ependymoma in childhood: prognostic factors, extent of surgery, and adjuvant therapy

Abstract

Overall survival, intraoperative deaths excluded, was 73 +/- 11% and 51 +/- 14% at 5 and 10 years, respectively. The event-free survival rate at 5 and 10 years was 48 +/- 12% and 46 +/- 12%, respectively. Most of the events were local recurrences. Despite multiple reinterventions, the overall survival rate in this group dropped to 14%. On univariate analysis, the only significant prognostic factors were complete resection and radiotherapy. Both of these factors combined increased the 5- and 10-year survival rates to 93 and 75%, respectively. Age of the patients was not a statistically independent prognostic factor. The patients in the chemotherapy group did not fare as well as those in the radiotherapy group. A subgroup (36%) within the chemotherapy group, however, survived tumor free after a mean follow-up period of 67 months. It is not clear whether this subgroup either responded well to chemotherapy or needed no adjuvant therapy. Further research is warranted to answer this question.

The prognostic value of histological grading of posterior fossa ependymomas in children: a Children's Oncology Group study and a review of prognostic factors

“…In many other studies, limited number of patients younger than 3 years might have been the reason for not finding age as an independent prognostic factor. 28 Another remote possibility may be that risk of young age could be more relevant for tumors in a particular location, 31 even though there are studies that reported age as a prognostic variable independent of tumor location. 4,25,34 It is possible to suggest that tumors in the very young may pose unique challenges in clinical management or they may possess more aggressive biological properties.…”

“…22 Tumor location was reported to significantly affect prognosis in six studies. [17][18][19]24,26,31 In all the studies with positive findings, posterior fossa tumors had a far less favorable outcome, but only three of these studies provided a multivariate analysis in which location still remained a significant prognostic factor 24,26,31 In the remaining 19 studies, location was either not analyzed as a prognostic factor or was found not to influence survival.…”

“…3,4,6,9,11,14,15,17,20,22,24,25,30 In some studies this ratio was almost equal, 13,19,[33][34][35] while in others there were far more Grade III than Grade II ependymomas. 12,26,28,29,31,32 In one study of ependymomas in children under 5 years of age, the authors reported eight Grade II and 60 Grade III ependymomas. 26 However, since none of the studies were apparently designed to provide prevalence of demographic or pathological features in a catchment area, these variations reflected a selection bias in location, age, or available material.…”

Pediatric intracranial ependymal tumors: clinicopathologic evaluation of 28 cases

“…Bu olgularda 5 yıllık yaşam süresi oranı tedavi protokollerine rağmen %70'i geçememektedir. Ependimomlarda prognozun kötü seyretmesinin en önemli nedeni tedaviye rağmen hastalığın olguların yaklaşık yarısında nüks etmesidir (3)(4)(5). Bu nedenle ependimomlarda tanı anında riskli hastaları saptayabilmek için, prognostik faktörleri araştıran çok sayıda araştırma yapılmıştır.…”

“…Bu nedenle ependimomlarda tanı anında riskli hastaları saptayabilmek için, prognostik faktörleri araştıran çok sayıda araştırma yapılmıştır. Yaş, cinsiyet, tümör lokalizasyonu, rezeksiyon tipi gibi klinik parametreler yanı sıra histolojik derece, Ki67 proliferatif indeksi ve p53 proteini gibi histopatolojik parametreler prognostik açıdan çeşitli araştırmalarda incelenmiş, tartışmalı sonuçlar bulunmuştur (2,4,(5)(6)(7)(8)(9)(10)(11)(12).…”