Relapsed intracranial ependymoma in children in the UK: Patterns of relapse, survival and therapeutic outcome

Messahel, Boo; Ashley, S.; Saran, Frank; Ellison, David W.; Ironside, James W.; Phipps, Kim; Cox, Tim; Chong, WK; Robinson, Kath; Picton, Sue; Pinkerton, CR; Mallucci, Conor; Macarthur, Donald; Jaspan, T.; Michalski, Antony; Grundy, Richard G.

doi:10.1016/j.ejca.2009.03.018

Cited by 77 publications

(63 citation statements)

References 42 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Despite these measures, the prognosis for pediatric intracranial ependymomas remains poor when compared with other childhood malignancies, with local tumor recurrence a frequently reported event, even after complete tumor excision (5). After 5 years, progression-free survival (PFS) rates range from 23% to 74% (3,9,10), whereas mortality is reported in up to 40% of affected children (3).…”

mentioning

confidence: 99%

Copy Number Gain of 1q25 Predicts Poor Progression-Free Survival for Pediatric Intracranial Ependymomas and Enables Patient Risk Stratification: A Prospective European Clinical Trial Cohort Analysis on Behalf of the Children's Cancer Leukaemia Group (CCLG), Société Française d'Oncologie Pédiatrique (SFOP), and International Society for Pediatric Oncology (SIOP)

Kilday

Mitra

Domerg

et al. 2012

Clinical Cancer Research

108

View full text Add to dashboard Cite

Purpose: The high incidence of recurrence and unpredictable clinical outcome for pediatric ependymoma reflect the imprecision of current therapeutic staging and need for novel risk stratification markers. We therefore evaluated 1q25 gain across three age-and treatment-defined European clinical trial cohorts of pediatric intracranial ependymoma.Experimental Design: Frequency of 1q gain was assessed across 48 ependymomas (42 primary, 6 recurrent) using Affymetrix 500K single-nucleotide polymorphism arrays. Gain of 1q25 was then evaluated by interphase FISH across 189 tumors treated on the Children's Cancer Leukaemia Group/International Society for Pediatric Oncology (SIOP) CNS9204 (n ¼ 60) and BBSFOP (n ¼ 65) adjuvant chemotherapy trials, or with primary postoperative radiotherapy (SIOP CNS9904/RT, n ¼ 64). Results were correlated with clinical, histologic, and survival data.Results: Gain of 1q was the most frequent imbalance in primary (7/42, 17%) and recurrent ependymomas (2/6, 33%). Gain of 1q25 was an independent predictor of tumor progression across the pooled trial cohort [HR ¼ 2.55; 95% confidence interval (CI): 1.56-4.16; P ¼ 0.0002] and both CNS9204 (HR ¼ 4.03; 95% CI: 1.88-8.63) and BBSFOP (HR ¼ 3.10; 95% CI: 1.22-7.86) groups. The only clinical variable associated with adverse outcome was incomplete tumor resection. Integrating tumor resectability with 1q25 status enabled stratification of cases into disease progression risk groups for all three trial cohorts.Conclusions: This is the first study to validate a prognostic genomic marker for childhood ependymoma across independent trial groups. 1q25 gain predicts disease progression and can contribute to patient risk stratification. We advocate the prospective evaluation of 1q25 gain as an adverse marker in future international clinical trials.

show abstract

mentioning

confidence: 99%

Copy Number Gain of 1q25 Predicts Poor Progression-Free Survival for Pediatric Intracranial Ependymomas and Enables Patient Risk Stratification: A Prospective European Clinical Trial Cohort Analysis on Behalf of the Children's Cancer Leukaemia Group (CCLG), Société Française d'Oncologie Pédiatrique (SFOP), and International Society for Pediatric Oncology (SIOP)

Kilday

Mitra

Domerg

et al. 2012

Clinical Cancer Research

108

View full text Add to dashboard Cite

show abstract

“…(3,6,10,11) Although chemotherapy has been found to delay progression in some cases and provides palliative relief, but has not been shown to increase survival. (11)(12)(13)(14) …”

Section: Discussionmentioning

confidence: 99%

Recurrent extraventricular anaplastic ependymoma with scalp metastasis

Agrawal¹,

Mohan²,

Santhi³

et al. 2014

Romanian Neurosurgery

View full text Add to dashboard Cite

Extraneural metastasis from anaplastic ependymoma is uncommon. In a study from Memorial Sloan Kettering Cancer Center where the authors reviewed 81 ependymomas cases (between 1956 and 1989) there were only five (6.2%) cases had extraneural metastases. We present a case of anaplastic ependymoma with scalp metastasis and discuss the possible mechanism of spread. In majority most of the cases of metastatic extracranial ependymoma patients have underlying progressive intracranial disease. Although these patients receive standard treatment for the primary tumor (Gross total resection and radiotherapy) and the management options for recurrences includes re-excision, focal reirradiation, stereotactic radiosurgery, or craniospinal radiotherapy for metastatic disease the long term outcome is not favorable.

show abstract

“…The relapse in the majority of patients with ependymoma in general and supratentorial ependymoma in particular, is local [15,15,25]. [5] 1957-1994 1-20 23 -65 Afra et al [28] 1958-1981 <15 46 -34.7 Vinchon et al [29] 1985-1999 <15 18 37 54 Robertson et al [15] 1986-1992 2-18 11 50 70 Horn et al [14] 1987-1991 <21 19 58 63 Hukin et al [4] 1990-1997 3-21 8 69 100 Grill et al [9] 1990-1998 <3 13 -100 Zacharoulis et al [30] 1991 [26]; all four patients with supratentorial ependymoma in their series who were treated with repeat surgery (gross total resection in three) and radiation therapy continued disease free after a median follow-up of 4 years.…”

Section: Discussionmentioning

confidence: 99%

Supratentorial ependymoma in children: To observe or to treat following gross total resection?

Venkatramani

Dhall

Patel

et al. 2011

Pediatric Blood & Cancer

View full text Add to dashboard Cite

BackgroundThe standard treatment for ependymoma is surgical resection followed by postoperative irradiation to the local site. The role of radiation therapy in completely resected supratentorial ependymoma has been questioned over the past two decades.ProcedureRetrospective review of the medical records of all consecutively diagnosed supratentorial ependymoma patients at Children's Hospital Los Angeles between January 1999 and December 2009.ResultsTen patients (three females) were included. The median age at presentation was 5.6 years (range 1.8–15.6 years). Reviewed histology was anaplastic ependymoma in seven patients and cellular ependymoma in three patients. Gross total resection was achieved in six patients; five were observed and one received chemotherapy. In the four patients who underwent subtotal resection, one was observed, two received local irradiation and one received irradiation and chemotherapy. The median length of follow up was 43 (range 22–81) months. Four relapses were observed; two patients who underwent initial gross total resection. All patients who underwent gross total resection were alive at the time of preparation of this article. The 5‐year progression‐free and overall survival rates were 53 ± 19% and 86 ± 13% respectively.ConclusionsRadiation therapy was avoided in five patients following gross total resection, four of whom had anaplastic histology. In some children with completely resected supratentorial ependymoma, surgery alone may be an acceptable treatment option. Pediatr Blood Cancer 2012; 58: 380–383. © 2011 Wiley Periodicals, Inc.

show abstract

Relapsed intracranial ependymoma in children in the UK: Patterns of relapse, survival and therapeutic outcome

Cited by 77 publications

References 42 publications

Recurrent extraventricular anaplastic ependymoma with scalp metastasis

Supratentorial ependymoma in children: To observe or to treat following gross total resection?

Contact Info

Product

Resources

About