Ependymoma
Rokowania, prognozy i postęp choroby
Rokowanie w wyściółczaku zależy od wielu czynników, w tym wieku pacjenta, stopnia złośliwości guza, zakresu resekcji oraz ekspresji markerów molekularnych takich jak hTERT i ABCB1. Pięcioletni wskaźnik przeżycia wynosi około 88,2% ogólnie, z gorszymi wynikami u pacjentów pediatrycznych (60%) w porównaniu do dorosłych (>65%). Dziesięcioletni wskaźnik przeżycia u dorosłych wynosi 70-89%, a u dzieci 50-70%. Całkowita resekcja guza i radioterapia znacząco poprawiają przeżycie wolne od progresji (PFS) i całkowite przeżycie (OS), zwłaszcza u dorosłych z wyściółczakami tylnego dołu czaszki, gdzie 10-letnie OS może sięgać 85%. Wiek i stopień usunięcia guza mają istotny wpływ na PFS (p=0,03) i OS (p=0,03), a guzy III stopnia cechują się gorszym rokowaniem. Ekspresja ABCB1 wiąże się z krótszym PFS (2,7 vs 8,6 lat; p=0,007) i OS (5,4 vs 12 lat; p=0,009).
- Rokowanie w przypadku wyściółczaka (ependymoma)
- Wskaźniki przeżycia w wyściółczaku
- Czynniki wpływające na rokowanie
- Rokowanie w zależności od stopnia resekcji
- Rokowanie w przypadku nawrotu choroby
- Markery molekularne jako czynniki prognostyczne
- Czynniki predykcyjne powrotu do zdrowia funkcjonalnego
- Strategia obserwacji po całkowitej resekcji
- Indywidualne podejście do rokowania
- Kolejne rozdziały
Rokowanie w przypadku wyściółczaka (ependymoma)
Rokowanie w przypadku wyściółczaka (ependymoma) stanowi przewidywanie prawdopodobnego przebiegu choroby i szans na wyleczenie, które lekarz przedstawia pacjentowi na podstawie dostępnych danych klinicznych i patologicznych. Należy pamiętać, że rokowanie jest procesem ciągłym i może ulegać zmianom na różnych etapach leczenia pacjenta1. Statystyki dotyczące przeżycia powinny być traktowane jako punkt odniesienia, a nie ostateczny wyznacznik indywidualnego przypadku2.
Wskaźniki przeżycia w wyściółczaku
Dane dotyczące przeżycia pacjentów z wyściółczakiem różnią się w zależności od wieku pacjenta, stopnia zaawansowania guza i innych czynników prognostycznych:
- Pięcioletni wskaźnik przeżycia dla wyściółczaka wynosi około 88,2%3
- U pacjentów pediatrycznych pięcioletni wskaźnik przeżycia wynosi około 60%45
- U dorosłych pacjentów pięcioletni wskaźnik przeżycia przekracza 65%, choć rokowanie jest gorsze w przypadku guzów III stopnia6
- Dziesięcioletni wskaźnik przeżycia u pacjentów dorosłych wynosi 70-89%7
- Dziesięcioletni wskaźnik całkowitego przeżycia u pacjentów pediatrycznych wynosi 64%8
- Ogólny dziesięcioletni wskaźnik przeżycia dla pediatrycznego wyściółczaka wynosi około 50-70% w Stanach Zjednoczonych9
W jednym z badań analizujących 33 pacjentów z wyściółczakiem wykazano, że dla sześciu pacjentów pediatrycznych przeżycie wolne od progresji choroby (PFS) wynosiło 43,7±13,5 miesięcy, a całkowite przeżycie (OS) 58,1±13,7 miesięcy. Dla 27 dorosłych pacjentów PFS wynosiło 125,6±14,3 miesięcy, a OS 151,2±12,5 miesięcy10.
Po całkowitej resekcji i radioterapii, dziesięcioletnie wskaźniki całkowitego przeżycia dla dorosłych pacjentów z wyściółczakami tylnego dołu czaszki zbliżają się do 85%11.
Czynniki wpływające na rokowanie
Wiele czynników wpływa na rokowanie w przypadku wyściółczaka:
- Wiek – jest istotnym czynnikiem prognostycznym, wykazującym statystycznie istotny wpływ na PFS (p=0,03) i OS (p=0,03). Dorośli pacjenci mają lepsze rokowanie niż dzieci1213
- Zakres resekcji – maksymalne bezpieczne usunięcie guza jest najlepszym leczeniem wyściółczaka. U dorosłych pacjentów stopień usunięcia guza znacząco wpływa na PFS (p=0,03) i wykazuje tendencję do wpływu na OS (p=0,06)1415
- Stopień złośliwości guza – guzy III stopnia mają gorsze rokowanie16
- Lokalizacja guza17
- Rozprzestrzenianie się guza – rokowanie jest lepsze, gdy guz nie rozprzestrzenia się na inne części mózgu lub rdzenia kręgowego18
- Ekspresja hTERT (enzymatycznej podjednostki telomerazy) – jest jednym z najważniejszych markerów prognostycznych; pacjenci z guzami niewykazującymi ekspresji hTERT mają lepsze rokowanie, nawet w przypadku nawrotu19
- Dysfunkcja telomerów – guzy wykazujące dysfunkcję telomerów (pozytywne dla H2AX) przy braku ekspresji hTERT mają lepsze rokowanie2021
- Ekspresja ABCB1 – pacjenci z wyściółczakiem wykazującym ekspresję ABCB1 mają krótsze średnie przeżycie wolne od zdarzeń (2,7 vs 8,6 lat; p=0,007) i całkowite przeżycie (5,4 vs 12 lat; p=0,009)22
Rokowanie w zależności od stopnia resekcji
Zakres resekcji guza jest jednym z najważniejszych czynników prognostycznych w wyściółczaku:
- Całkowicie usunięte wyściółczaki u dorosłych bez cech anaplazji mogą być obserwowane bez dodatkowego leczenia, niezależnie od lokalizacji guza2324
- W jednym badaniu tylko jeden z 12 pacjentów, u których całkowicie usunięto wyściółczaka bez cech anaplazji, rozwinął miejscową wznowę25
- Większy zakres resekcji i adjuwantowa radioterapia znacząco poprawiają PFS u dorosłych pacjentów z wyściółczakami tylnego dołu czaszki26
- U pacjentów z niecałkowicie usuniętym guzem, ekspresja ABCB1 koreluje ze zmniejszonym przeżyciem wolnym od zdarzeń (hazard ratio 2,64, 95% CI 1,22-5,73)27
Rokowanie w przypadku nawrotu choroby
Nawrót wyściółczaka znacząco pogarsza rokowanie:
- Rokowanie w przypadku nawrotu jest niekorzystne – tylko 25% dzieci przeżywa28
- Późne nawroty (po ponad 5 latach) mogą występować u dzieci z niektórymi typami wyściółczaka29
- Guzy mają tendencję do ewoluowania w kierunku bardziej agresywnego fenotypu biologicznego podczas nawrotu30
- Zawsze istnieje możliwość wznowy wyściółczaka, nawet po uznaniu pacjenta za wolnego od nowotworu, co jest bardziej prawdopodobne w przypadku guzów III stopnia31
Markery molekularne jako czynniki prognostyczne
Odkrycie znaczenia markerów molekularnych przyczynia się do lepszego przewidywania przebiegu choroby:
- hTERT (podjednostka telomerazy) – jest najważniejszym pojedynczym markerem prognostycznym wśród znanych czynników patologicznych i klinicznych w momencie diagnozy32
- Dysfunkcja telomerów (pozytywność H2AX) – uderzająca różnica w PFS między morfologicznie podobnymi guzami, które nie wykazują ekspresji hTERT i wykazują dysfunkcję telomerów, a guzami które wykazują ekspresję hTERT i mają funkcjonalne telomery33
- ABCB1 – marker predykcyjny odpowiedzi na chemioterapię u pacjentów z wyściółczakiem; ekspresja ABCB1 koreluje z niższym 5-letnim przeżyciem wolnym od zdarzeń (13% vs 50%) i niższym 5-letnim całkowitym przeżyciem (33% vs 74%)34
Czynniki predykcyjne powrotu do zdrowia funkcjonalnego
W przypadku wyściółczaków tylnego dołu czaszki u dorosłych zidentyfikowano niezależne czynniki predykcyjne tempa powrotu do zdrowia funkcjonalnego:
- Wielkość guza
- Zmiany torbielowate
- Potrzeba odprowadzania płynu mózgowo-rdzeniowego
Te predyktory wyniku funkcjonalnego mogą kierować przedoperacyjnymi szacunkami powrotu do zdrowia po mikrochirurgicznej resekcji35.
Strategia obserwacji po całkowitej resekcji
Badania sugerują, że całkowicie usunięte wyściółczaki II stopnia u dorosłych mogą być monitorowane bez jakiegokolwiek leczenia adjuwantowego, niezależnie od lokalizacji guza:
- Polityka „czekaj i obserwuj” może być zalecana w całkowicie usuniętych wyściółczakach II stopnia według WHO u dorosłych, niezależnie od lokalizacji guza36
- Dodatkowe leczenie może być potrzebne, gdy obserwuje się nawrót po radiologicznej obserwacji37
Indywidualne podejście do rokowania
Rokowanie w wyściółczaku musi być zawsze rozpatrywane indywidualnie:
- Statystyki i średnie nie mogą określić, co stanie się w konkretnym przypadku38
- Wartość wskaźników przeżycia może nie mieć zastosowania do indywidualnej sytuacji pacjenta39
- Lekarz prowadzący jest najlepszym źródłem informacji na temat rokowania40
- Rokowanie zależy od wielu czynników, a każdy przypadek jest inny41
Choć nie ma lekarstwa na raka, możliwe jest usunięcie lub zniszczenie wyściółczaka III stopnia za pomocą leczenia42. Wyściółczak jest trudny do wyleczenia bez udanej operacji usunięcia guza. Jeśli operacja jest niekompletna lub wyściółczak nawraca, długoterminowe rokowanie jest zwykle niekorzystne43.
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Materiały źródłowe
- #1 Ependymoma Prognosis | Brain Tumour Survival Rateshttps://www.thebraintumourcharity.org/brain-tumour-diagnosis-treatment/types-brain-tumour-children/ependymoma/ependymoma-prognosis/
A prognosis is when your doctor gives you an indication of the likely outcome of your medical condition. […] When discussing ependymoma prognosis, your doctor or medical team will tell you what they think will happen following your treatment. […] But, keep in mind that your doctor cant be absolutely certain about what will happen to you following a diagnosis of an ependymoma. […] They can give you an estimate, based on your tumour type and current situation. […] This is why ependymoma prognosis is often an ongoing process. And, it might change at different stages in your journey. […] About 60% of children diagnosed with an ependymoma brain tumour survive for five years or more. […] It is important to remember that statistics and averages cannot tell you what will happen to you specifically.
- #2 Prognosis | CERN Foundationhttps://www.cern-foundation.org/education/diagnosis/prognosis
Prognosis statistics should be used as a reference, but not a final predictor of your specific case. […] The five-year survival rate for pediatric patients with ependymoma is roughly 60 percent. […] According to multiple studies, the five-year survival rate for adult ependymoma is over 65 percent, although the prognosis is worse for patients with grade III tumors. […] Overall, prognosis is improved when the entire tumor can be removed by surgery and if there is no spread of the tumor to other parts of the brain or spinal cord.
- #3 Ependymoma: Diagnosis and Treatment – NCIhttps://www.cancer.gov/rare-brain-spine-tumor/tumors/ependymoma
The likely outcome of the disease or chance of recovery is called prognosis. Prognosis is based on the tumor grade, location, tumor type, extent of tumor spread, genetic findings, patients age, and tumor remaining after surgery (if surgery is possible). […] The relative five-year survival rate for ependymoma is 88.2 percent. However, many factors can affect prognosis. These include the tumor grade and molecular type, the persons age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.
- #4 Ependymoma Prognosis | Brain Tumour Survival Rateshttps://www.thebraintumourcharity.org/brain-tumour-diagnosis-treatment/types-brain-tumour-children/ependymoma/ependymoma-prognosis/
A prognosis is when your doctor gives you an indication of the likely outcome of your medical condition. […] When discussing ependymoma prognosis, your doctor or medical team will tell you what they think will happen following your treatment. […] But, keep in mind that your doctor cant be absolutely certain about what will happen to you following a diagnosis of an ependymoma. […] They can give you an estimate, based on your tumour type and current situation. […] This is why ependymoma prognosis is often an ongoing process. And, it might change at different stages in your journey. […] About 60% of children diagnosed with an ependymoma brain tumour survive for five years or more. […] It is important to remember that statistics and averages cannot tell you what will happen to you specifically.
- #5 Prognosis | CERN Foundationhttps://www.cern-foundation.org/education/diagnosis/prognosis
Prognosis statistics should be used as a reference, but not a final predictor of your specific case. […] The five-year survival rate for pediatric patients with ependymoma is roughly 60 percent. […] According to multiple studies, the five-year survival rate for adult ependymoma is over 65 percent, although the prognosis is worse for patients with grade III tumors. […] Overall, prognosis is improved when the entire tumor can be removed by surgery and if there is no spread of the tumor to other parts of the brain or spinal cord.
- #6 Prognosis | CERN Foundationhttps://www.cern-foundation.org/education/diagnosis/prognosis
Prognosis statistics should be used as a reference, but not a final predictor of your specific case. […] The five-year survival rate for pediatric patients with ependymoma is roughly 60 percent. […] According to multiple studies, the five-year survival rate for adult ependymoma is over 65 percent, although the prognosis is worse for patients with grade III tumors. […] Overall, prognosis is improved when the entire tumor can be removed by surgery and if there is no spread of the tumor to other parts of the brain or spinal cord.
- #7 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The 10-year survival rate for adult patients is 7089% and the 10-year overall survival rate is 64% in pediatric patients with ependymomas. […] Age and the extent of surgery were considered as the major predictors of outcome in patients with ependymomas. Adult patients have better prognosis than children. […] Maximal safe resection is the best treatment for ependymoma. […] In this study, we focused on the prognosis of totally resected adult ependymomas without anaplastic pathology and postoperative adjuvant treatment. […] In our study, only one out of the 12 patients who had totally resected ependymomas without anaplastic pathology developed local recurrence. […] This suggests that additional treatment could be needed when recurrence is observed after radiological follow-up. A wait and see policy could be recommended in totally resected WHO grade II adult ependymomas, regardless of the tumor location. […] In conclusion, we analyzed 33 ependymoma patients for prognostic factors. Age is an important factor of prognosis. In adult patients, totally resected adult ependymomas without anaplastic pathology could be monitored without any adjuvant treatment, regardless of the tumor location.
- #8 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The 10-year survival rate for adult patients is 7089% and the 10-year overall survival rate is 64% in pediatric patients with ependymomas. […] Age and the extent of surgery were considered as the major predictors of outcome in patients with ependymomas. Adult patients have better prognosis than children. […] Maximal safe resection is the best treatment for ependymoma. […] In this study, we focused on the prognosis of totally resected adult ependymomas without anaplastic pathology and postoperative adjuvant treatment. […] In our study, only one out of the 12 patients who had totally resected ependymomas without anaplastic pathology developed local recurrence. […] This suggests that additional treatment could be needed when recurrence is observed after radiological follow-up. A wait and see policy could be recommended in totally resected WHO grade II adult ependymomas, regardless of the tumor location. […] In conclusion, we analyzed 33 ependymoma patients for prognostic factors. Age is an important factor of prognosis. In adult patients, totally resected adult ependymomas without anaplastic pathology could be monitored without any adjuvant treatment, regardless of the tumor location.
- #9 Ependymoma in Children and Teens – Together by St. Judeâ¢https://together.stjude.org/en-us/conditions/cancers/ependymoma.html
The overall 10-year survival rate for pediatric ependymoma is about 5070% (57 of 10) in the United States. […] Factors that affect the chance of cure include: Location of the tumor, Type and grade of the tumor, If the cancer has spread to other parts of the body, If surgery can completely remove the tumor, Age at diagnosis, If the cancer has come back after treatment, Molecular features of the tumor. […] Children with certain types of ependymoma tend to have a better chance for long-term survival. But late recurrences or relapse (beyond 5 years) may occur. […] Ependymoma is hard to cure without successful surgery to remove the tumor. If surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor. […] Prognosis for ependymoma depends on many factors. Every case is different. Always talk to your care team about your childs case. […] Your childs doctor is the best source of information about prognosis.
- #10 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The purpose of this study was to evaluate the prognostic factors and outcomes in patients with ependymoma to management plans. […] For six pediatric patients, the progression-free survival (PFS) was 43.713.5 months, and the overall survival (OS) was 58.113.7 months. For 27 adult patients, the PFS was 125.614.3 months, and the OS was 151.212.5 months. Age demonstrated a statistically significant effect on PFS (p=0.03) and OS (p=0.03). In adult ependymomas, the extent of tumor removal significantly affected PFS (p=0.03) and trended towards an effect on OS (p=0.06). […] Adult patients with ependymomas were found to have better survival rates compared to pediatric patients. We suggest that totally resected adult ependymomas without anaplastic pathology could be observed without any adjuvant treatment, regardless of the tumor location.
- #11 Predictors of functional recovery in adults with posterior fossa ependymomas – PubMedhttps://pubmed.ncbi.nlm.nih.gov/24579660/
After complete resection and radiation therapy, the 10-year overall survival rates for adult patients with posterior fossa ependymomas approach 85%. […] Greater extent of resection and adjuvant radiotherapy significantly improve PFS in adult patients with posterior fossa ependymomas. Tumor size, cystic changes, and the need for CSF diversion were independent predictors of the rate of functional recovery in this patient population. […] These functional outcome predictors may guide preoperative estimations of recovery following microsurgical resection.
- #12 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The purpose of this study was to evaluate the prognostic factors and outcomes in patients with ependymoma to management plans. […] For six pediatric patients, the progression-free survival (PFS) was 43.713.5 months, and the overall survival (OS) was 58.113.7 months. For 27 adult patients, the PFS was 125.614.3 months, and the OS was 151.212.5 months. Age demonstrated a statistically significant effect on PFS (p=0.03) and OS (p=0.03). In adult ependymomas, the extent of tumor removal significantly affected PFS (p=0.03) and trended towards an effect on OS (p=0.06). […] Adult patients with ependymomas were found to have better survival rates compared to pediatric patients. We suggest that totally resected adult ependymomas without anaplastic pathology could be observed without any adjuvant treatment, regardless of the tumor location.
- #13 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The 10-year survival rate for adult patients is 7089% and the 10-year overall survival rate is 64% in pediatric patients with ependymomas. […] Age and the extent of surgery were considered as the major predictors of outcome in patients with ependymomas. Adult patients have better prognosis than children. […] Maximal safe resection is the best treatment for ependymoma. […] In this study, we focused on the prognosis of totally resected adult ependymomas without anaplastic pathology and postoperative adjuvant treatment. […] In our study, only one out of the 12 patients who had totally resected ependymomas without anaplastic pathology developed local recurrence. […] This suggests that additional treatment could be needed when recurrence is observed after radiological follow-up. A wait and see policy could be recommended in totally resected WHO grade II adult ependymomas, regardless of the tumor location. […] In conclusion, we analyzed 33 ependymoma patients for prognostic factors. Age is an important factor of prognosis. In adult patients, totally resected adult ependymomas without anaplastic pathology could be monitored without any adjuvant treatment, regardless of the tumor location.
- #14 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The purpose of this study was to evaluate the prognostic factors and outcomes in patients with ependymoma to management plans. […] For six pediatric patients, the progression-free survival (PFS) was 43.713.5 months, and the overall survival (OS) was 58.113.7 months. For 27 adult patients, the PFS was 125.614.3 months, and the OS was 151.212.5 months. Age demonstrated a statistically significant effect on PFS (p=0.03) and OS (p=0.03). In adult ependymomas, the extent of tumor removal significantly affected PFS (p=0.03) and trended towards an effect on OS (p=0.06). […] Adult patients with ependymomas were found to have better survival rates compared to pediatric patients. We suggest that totally resected adult ependymomas without anaplastic pathology could be observed without any adjuvant treatment, regardless of the tumor location.
- #15 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The 10-year survival rate for adult patients is 7089% and the 10-year overall survival rate is 64% in pediatric patients with ependymomas. […] Age and the extent of surgery were considered as the major predictors of outcome in patients with ependymomas. Adult patients have better prognosis than children. […] Maximal safe resection is the best treatment for ependymoma. […] In this study, we focused on the prognosis of totally resected adult ependymomas without anaplastic pathology and postoperative adjuvant treatment. […] In our study, only one out of the 12 patients who had totally resected ependymomas without anaplastic pathology developed local recurrence. […] This suggests that additional treatment could be needed when recurrence is observed after radiological follow-up. A wait and see policy could be recommended in totally resected WHO grade II adult ependymomas, regardless of the tumor location. […] In conclusion, we analyzed 33 ependymoma patients for prognostic factors. Age is an important factor of prognosis. In adult patients, totally resected adult ependymomas without anaplastic pathology could be monitored without any adjuvant treatment, regardless of the tumor location.
- #16 Prognosis | CERN Foundationhttps://www.cern-foundation.org/education/diagnosis/prognosis
Prognosis statistics should be used as a reference, but not a final predictor of your specific case. […] The five-year survival rate for pediatric patients with ependymoma is roughly 60 percent. […] According to multiple studies, the five-year survival rate for adult ependymoma is over 65 percent, although the prognosis is worse for patients with grade III tumors. […] Overall, prognosis is improved when the entire tumor can be removed by surgery and if there is no spread of the tumor to other parts of the brain or spinal cord.
- #17 Ependymoma in Children and Teens – Together by St. Judeâ¢https://together.stjude.org/en-us/conditions/cancers/ependymoma.html
The overall 10-year survival rate for pediatric ependymoma is about 5070% (57 of 10) in the United States. […] Factors that affect the chance of cure include: Location of the tumor, Type and grade of the tumor, If the cancer has spread to other parts of the body, If surgery can completely remove the tumor, Age at diagnosis, If the cancer has come back after treatment, Molecular features of the tumor. […] Children with certain types of ependymoma tend to have a better chance for long-term survival. But late recurrences or relapse (beyond 5 years) may occur. […] Ependymoma is hard to cure without successful surgery to remove the tumor. If surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor. […] Prognosis for ependymoma depends on many factors. Every case is different. Always talk to your care team about your childs case. […] Your childs doctor is the best source of information about prognosis.
- #18 Prognosis | CERN Foundationhttps://www.cern-foundation.org/education/diagnosis/prognosis
Prognosis statistics should be used as a reference, but not a final predictor of your specific case. […] The five-year survival rate for pediatric patients with ependymoma is roughly 60 percent. […] According to multiple studies, the five-year survival rate for adult ependymoma is over 65 percent, although the prognosis is worse for patients with grade III tumors. […] Overall, prognosis is improved when the entire tumor can be removed by surgery and if there is no spread of the tumor to other parts of the brain or spinal cord.
- #19 Telomere maintenance and dysfunction predict recurrence in paediatric ependymoma | British Journal of Cancerhttps://www.nature.com/articles/6604652
We have recently described the enzymatic subunit of telomerase (hTERT) as an important prognostic marker for paediatric ependymoma. […] This study emphasises the importance of telomere biology as a prognostic tool and telomerase inhibition as a therapeutic target for paediatric ependymoma. […] To address this problem, we recently reported that the expression of hTERT, the enzymatic subunit of human telomerase, is the single most important predictor of outcome among known pathological and clinical risk factors at diagnosis. […] The biological importance of our findings is supported by the prognostic significance conferred by the demonstration of telomere maintenance (hTERT expression) and lack of telomere dysfunction (H2AX negativity) in paediatric ependymomas. […] Taken together, these findings support the concept that tumours tend to evolve towards a more aggressive biological phenotype at relapse. However, interestingly tumours that continued to lack hTERT expression had a better OS even at recurrence (log rank P=0.05).
- #20 Telomere maintenance and dysfunction predict recurrence in paediatric ependymoma | British Journal of Cancerhttps://www.nature.com/articles/6604652
We have recently described the enzymatic subunit of telomerase (hTERT) as an important prognostic marker for paediatric ependymoma. […] This study emphasises the importance of telomere biology as a prognostic tool and telomerase inhibition as a therapeutic target for paediatric ependymoma. […] To address this problem, we recently reported that the expression of hTERT, the enzymatic subunit of human telomerase, is the single most important predictor of outcome among known pathological and clinical risk factors at diagnosis. […] The biological importance of our findings is supported by the prognostic significance conferred by the demonstration of telomere maintenance (hTERT expression) and lack of telomere dysfunction (H2AX negativity) in paediatric ependymomas. […] Taken together, these findings support the concept that tumours tend to evolve towards a more aggressive biological phenotype at relapse. However, interestingly tumours that continued to lack hTERT expression had a better OS even at recurrence (log rank P=0.05).
- #21 Telomere maintenance and dysfunction predict recurrence in paediatric ependymoma | British Journal of Cancerhttps://www.nature.com/articles/6604652
To determine whether proliferative markers that correlate with hTERT expression could predict outcome, we analysed each marker separately. […] The striking difference in PFS between morphologically similar tumours, which lack hTERT expression and show telomere dysfunction (H2AX positivity) on the one hand, and tumours that express hTERT and have functional telomeres (H2AX()) on the other, may allow for a reevaluation of tailored management for these patients.
- #22 A role for ABCB1 in prognosis, invasion and drug resistance in ependymoma | Scientific Reportshttps://www.nature.com/articles/s41598-019-46700-z
Three of the hallmarks of poor prognosis in paediatric ependymoma are drug resistance, local invasion and recurrence. […] We demonstrate that ABCB1 positive patients from an infant chemotherapy-led trial (CNS9204) had a shorter mean event free survival (EFS) (2.7 versus 8.6 years; p=0.007 log-rank analysis) and overall survival (OS) (5.4 versus 12 years; p=0.009 log-rank analysis). […] ABCB1 positivity also correlated with reduced event free survival in patients with incompletely resected tumours who received chemotherapy across CNS9204 and CNS9904 (a radiotherapy-led SIOP 1999-04 trial cohort; p=0.03). […] ABCB1 is a predictive marker of chemotherapy response in ependymoma patients and vardenafil, currently used to treat paediatric pulmonary hypertension in children, could be repurposed to reduce chemoresistance, migration and invasion in paediatric ependymoma patients at non-toxic concentrations.
- #23 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The purpose of this study was to evaluate the prognostic factors and outcomes in patients with ependymoma to management plans. […] For six pediatric patients, the progression-free survival (PFS) was 43.713.5 months, and the overall survival (OS) was 58.113.7 months. For 27 adult patients, the PFS was 125.614.3 months, and the OS was 151.212.5 months. Age demonstrated a statistically significant effect on PFS (p=0.03) and OS (p=0.03). In adult ependymomas, the extent of tumor removal significantly affected PFS (p=0.03) and trended towards an effect on OS (p=0.06). […] Adult patients with ependymomas were found to have better survival rates compared to pediatric patients. We suggest that totally resected adult ependymomas without anaplastic pathology could be observed without any adjuvant treatment, regardless of the tumor location.
- #24 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The 10-year survival rate for adult patients is 7089% and the 10-year overall survival rate is 64% in pediatric patients with ependymomas. […] Age and the extent of surgery were considered as the major predictors of outcome in patients with ependymomas. Adult patients have better prognosis than children. […] Maximal safe resection is the best treatment for ependymoma. […] In this study, we focused on the prognosis of totally resected adult ependymomas without anaplastic pathology and postoperative adjuvant treatment. […] In our study, only one out of the 12 patients who had totally resected ependymomas without anaplastic pathology developed local recurrence. […] This suggests that additional treatment could be needed when recurrence is observed after radiological follow-up. A wait and see policy could be recommended in totally resected WHO grade II adult ependymomas, regardless of the tumor location. […] In conclusion, we analyzed 33 ependymoma patients for prognostic factors. Age is an important factor of prognosis. In adult patients, totally resected adult ependymomas without anaplastic pathology could be monitored without any adjuvant treatment, regardless of the tumor location.
- #25 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The 10-year survival rate for adult patients is 7089% and the 10-year overall survival rate is 64% in pediatric patients with ependymomas. […] Age and the extent of surgery were considered as the major predictors of outcome in patients with ependymomas. Adult patients have better prognosis than children. […] Maximal safe resection is the best treatment for ependymoma. […] In this study, we focused on the prognosis of totally resected adult ependymomas without anaplastic pathology and postoperative adjuvant treatment. […] In our study, only one out of the 12 patients who had totally resected ependymomas without anaplastic pathology developed local recurrence. […] This suggests that additional treatment could be needed when recurrence is observed after radiological follow-up. A wait and see policy could be recommended in totally resected WHO grade II adult ependymomas, regardless of the tumor location. […] In conclusion, we analyzed 33 ependymoma patients for prognostic factors. Age is an important factor of prognosis. In adult patients, totally resected adult ependymomas without anaplastic pathology could be monitored without any adjuvant treatment, regardless of the tumor location.
- #26 Predictors of functional recovery in adults with posterior fossa ependymomas – PubMedhttps://pubmed.ncbi.nlm.nih.gov/24579660/
After complete resection and radiation therapy, the 10-year overall survival rates for adult patients with posterior fossa ependymomas approach 85%. […] Greater extent of resection and adjuvant radiotherapy significantly improve PFS in adult patients with posterior fossa ependymomas. Tumor size, cystic changes, and the need for CSF diversion were independent predictors of the rate of functional recovery in this patient population. […] These functional outcome predictors may guide preoperative estimations of recovery following microsurgical resection.
- #27 A role for ABCB1 in prognosis, invasion and drug resistance in ependymoma | Scientific Reportshttps://www.nature.com/articles/s41598-019-46700-z
The prognosis at relapse is dismal, with only 25% of children surviving. […] These tumours tend to invade surrounding critical structures such as the brain stem, making complete surgical resection difficult and relapse more likely. […] In the CNS9204 trial cohort, outcome for ABCB1 positive-patients correlated with lower 5 year event free survival (EFS; 13% versus 50%) P=0.007 and lower 5 year overall survival (OS; 33% versus 74%) p=0.009 (log-rank analysis). […] The correlation with outcome held in Cox Regression multivariate analysis where ABCB1 was an independent factor, even after adjustment for resection status and grade, for EFS (Hazard Ratio 2.8 confidence interval 1.35-5.9) p=0.007 and OS (Hazard Ratio 3.0 confidence interval 1.3-6.8) p=0.008. […] ABCB1 positive patients are more likely to relapse, in particular those who had incomplete tumour resection (hazard ratio 2.64, 95% CI 1.22-5.73). […] ABCB1 expression in a small sub-population of tumour cells is able to confer a drug resistant phenotype in paediatric ependymoma patients in CNS9204.
- #28 A role for ABCB1 in prognosis, invasion and drug resistance in ependymoma | Scientific Reportshttps://www.nature.com/articles/s41598-019-46700-z
The prognosis at relapse is dismal, with only 25% of children surviving. […] These tumours tend to invade surrounding critical structures such as the brain stem, making complete surgical resection difficult and relapse more likely. […] In the CNS9204 trial cohort, outcome for ABCB1 positive-patients correlated with lower 5 year event free survival (EFS; 13% versus 50%) P=0.007 and lower 5 year overall survival (OS; 33% versus 74%) p=0.009 (log-rank analysis). […] The correlation with outcome held in Cox Regression multivariate analysis where ABCB1 was an independent factor, even after adjustment for resection status and grade, for EFS (Hazard Ratio 2.8 confidence interval 1.35-5.9) p=0.007 and OS (Hazard Ratio 3.0 confidence interval 1.3-6.8) p=0.008. […] ABCB1 positive patients are more likely to relapse, in particular those who had incomplete tumour resection (hazard ratio 2.64, 95% CI 1.22-5.73). […] ABCB1 expression in a small sub-population of tumour cells is able to confer a drug resistant phenotype in paediatric ependymoma patients in CNS9204.
- #29 Ependymoma in Children and Teens – Together by St. Judeâ¢https://together.stjude.org/en-us/conditions/cancers/ependymoma.html
The overall 10-year survival rate for pediatric ependymoma is about 5070% (57 of 10) in the United States. […] Factors that affect the chance of cure include: Location of the tumor, Type and grade of the tumor, If the cancer has spread to other parts of the body, If surgery can completely remove the tumor, Age at diagnosis, If the cancer has come back after treatment, Molecular features of the tumor. […] Children with certain types of ependymoma tend to have a better chance for long-term survival. But late recurrences or relapse (beyond 5 years) may occur. […] Ependymoma is hard to cure without successful surgery to remove the tumor. If surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor. […] Prognosis for ependymoma depends on many factors. Every case is different. Always talk to your care team about your childs case. […] Your childs doctor is the best source of information about prognosis.
- #30 Telomere maintenance and dysfunction predict recurrence in paediatric ependymoma | British Journal of Cancerhttps://www.nature.com/articles/6604652
We have recently described the enzymatic subunit of telomerase (hTERT) as an important prognostic marker for paediatric ependymoma. […] This study emphasises the importance of telomere biology as a prognostic tool and telomerase inhibition as a therapeutic target for paediatric ependymoma. […] To address this problem, we recently reported that the expression of hTERT, the enzymatic subunit of human telomerase, is the single most important predictor of outcome among known pathological and clinical risk factors at diagnosis. […] The biological importance of our findings is supported by the prognostic significance conferred by the demonstration of telomere maintenance (hTERT expression) and lack of telomere dysfunction (H2AX negativity) in paediatric ependymomas. […] Taken together, these findings support the concept that tumours tend to evolve towards a more aggressive biological phenotype at relapse. However, interestingly tumours that continued to lack hTERT expression had a better OS even at recurrence (log rank P=0.05).
- #31 Ependymoma: Symptoms, Treatment, Prognosis & Typeshttps://my.clevelandclinic.org/health/diseases/23147-ependymoma
The five-year survival rate for ependymomas is nearly 85%. That means around 85% of people are still alive within five years of being diagnosed. But this number may not apply to you or your unique situation. Your providers will tell you what can expect. […] Theres no cure for cancer, but its possible to remove or destroy a grade 3 ependymoma with treatment. […] Remember, theres always a chance an ependymoma regrows (recurs), even after youve been declared cancer-free. Thats much more likely with grade 3 tumors. Your providers and surgeon will tell you what to expect based on your age and overall health.
- #32 Telomere maintenance and dysfunction predict recurrence in paediatric ependymoma | British Journal of Cancerhttps://www.nature.com/articles/6604652
We have recently described the enzymatic subunit of telomerase (hTERT) as an important prognostic marker for paediatric ependymoma. […] This study emphasises the importance of telomere biology as a prognostic tool and telomerase inhibition as a therapeutic target for paediatric ependymoma. […] To address this problem, we recently reported that the expression of hTERT, the enzymatic subunit of human telomerase, is the single most important predictor of outcome among known pathological and clinical risk factors at diagnosis. […] The biological importance of our findings is supported by the prognostic significance conferred by the demonstration of telomere maintenance (hTERT expression) and lack of telomere dysfunction (H2AX negativity) in paediatric ependymomas. […] Taken together, these findings support the concept that tumours tend to evolve towards a more aggressive biological phenotype at relapse. However, interestingly tumours that continued to lack hTERT expression had a better OS even at recurrence (log rank P=0.05).
- #33 Telomere maintenance and dysfunction predict recurrence in paediatric ependymoma | British Journal of Cancerhttps://www.nature.com/articles/6604652
To determine whether proliferative markers that correlate with hTERT expression could predict outcome, we analysed each marker separately. […] The striking difference in PFS between morphologically similar tumours, which lack hTERT expression and show telomere dysfunction (H2AX positivity) on the one hand, and tumours that express hTERT and have functional telomeres (H2AX()) on the other, may allow for a reevaluation of tailored management for these patients.
- #34 A role for ABCB1 in prognosis, invasion and drug resistance in ependymoma | Scientific Reportshttps://www.nature.com/articles/s41598-019-46700-z
The prognosis at relapse is dismal, with only 25% of children surviving. […] These tumours tend to invade surrounding critical structures such as the brain stem, making complete surgical resection difficult and relapse more likely. […] In the CNS9204 trial cohort, outcome for ABCB1 positive-patients correlated with lower 5 year event free survival (EFS; 13% versus 50%) P=0.007 and lower 5 year overall survival (OS; 33% versus 74%) p=0.009 (log-rank analysis). […] The correlation with outcome held in Cox Regression multivariate analysis where ABCB1 was an independent factor, even after adjustment for resection status and grade, for EFS (Hazard Ratio 2.8 confidence interval 1.35-5.9) p=0.007 and OS (Hazard Ratio 3.0 confidence interval 1.3-6.8) p=0.008. […] ABCB1 positive patients are more likely to relapse, in particular those who had incomplete tumour resection (hazard ratio 2.64, 95% CI 1.22-5.73). […] ABCB1 expression in a small sub-population of tumour cells is able to confer a drug resistant phenotype in paediatric ependymoma patients in CNS9204.
- #35 Predictors of functional recovery in adults with posterior fossa ependymomas – PubMedhttps://pubmed.ncbi.nlm.nih.gov/24579660/
After complete resection and radiation therapy, the 10-year overall survival rates for adult patients with posterior fossa ependymomas approach 85%. […] Greater extent of resection and adjuvant radiotherapy significantly improve PFS in adult patients with posterior fossa ependymomas. Tumor size, cystic changes, and the need for CSF diversion were independent predictors of the rate of functional recovery in this patient population. […] These functional outcome predictors may guide preoperative estimations of recovery following microsurgical resection.
- #36 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The 10-year survival rate for adult patients is 7089% and the 10-year overall survival rate is 64% in pediatric patients with ependymomas. […] Age and the extent of surgery were considered as the major predictors of outcome in patients with ependymomas. Adult patients have better prognosis than children. […] Maximal safe resection is the best treatment for ependymoma. […] In this study, we focused on the prognosis of totally resected adult ependymomas without anaplastic pathology and postoperative adjuvant treatment. […] In our study, only one out of the 12 patients who had totally resected ependymomas without anaplastic pathology developed local recurrence. […] This suggests that additional treatment could be needed when recurrence is observed after radiological follow-up. A wait and see policy could be recommended in totally resected WHO grade II adult ependymomas, regardless of the tumor location. […] In conclusion, we analyzed 33 ependymoma patients for prognostic factors. Age is an important factor of prognosis. In adult patients, totally resected adult ependymomas without anaplastic pathology could be monitored without any adjuvant treatment, regardless of the tumor location.
- #37 Ependymomas: Prognostic Factors and Outcome Analysis in a Retrospective Series of 33 Patientshttps://pmc.ncbi.nlm.nih.gov/articles/PMC5700030/
The 10-year survival rate for adult patients is 7089% and the 10-year overall survival rate is 64% in pediatric patients with ependymomas. […] Age and the extent of surgery were considered as the major predictors of outcome in patients with ependymomas. Adult patients have better prognosis than children. […] Maximal safe resection is the best treatment for ependymoma. […] In this study, we focused on the prognosis of totally resected adult ependymomas without anaplastic pathology and postoperative adjuvant treatment. […] In our study, only one out of the 12 patients who had totally resected ependymomas without anaplastic pathology developed local recurrence. […] This suggests that additional treatment could be needed when recurrence is observed after radiological follow-up. A wait and see policy could be recommended in totally resected WHO grade II adult ependymomas, regardless of the tumor location. […] In conclusion, we analyzed 33 ependymoma patients for prognostic factors. Age is an important factor of prognosis. In adult patients, totally resected adult ependymomas without anaplastic pathology could be monitored without any adjuvant treatment, regardless of the tumor location.
- #38 Ependymoma Prognosis | Brain Tumour Survival Rateshttps://www.thebraintumourcharity.org/brain-tumour-diagnosis-treatment/types-brain-tumour-children/ependymoma/ependymoma-prognosis/
A prognosis is when your doctor gives you an indication of the likely outcome of your medical condition. […] When discussing ependymoma prognosis, your doctor or medical team will tell you what they think will happen following your treatment. […] But, keep in mind that your doctor cant be absolutely certain about what will happen to you following a diagnosis of an ependymoma. […] They can give you an estimate, based on your tumour type and current situation. […] This is why ependymoma prognosis is often an ongoing process. And, it might change at different stages in your journey. […] About 60% of children diagnosed with an ependymoma brain tumour survive for five years or more. […] It is important to remember that statistics and averages cannot tell you what will happen to you specifically.
- #39 Ependymoma: Symptoms, Treatment, Prognosis & Typeshttps://my.clevelandclinic.org/health/diseases/23147-ependymoma
The five-year survival rate for ependymomas is nearly 85%. That means around 85% of people are still alive within five years of being diagnosed. But this number may not apply to you or your unique situation. Your providers will tell you what can expect. […] Theres no cure for cancer, but its possible to remove or destroy a grade 3 ependymoma with treatment. […] Remember, theres always a chance an ependymoma regrows (recurs), even after youve been declared cancer-free. Thats much more likely with grade 3 tumors. Your providers and surgeon will tell you what to expect based on your age and overall health.
- #40 Ependymoma in Children and Teens – Together by St. Judeâ¢https://together.stjude.org/en-us/conditions/cancers/ependymoma.html
The overall 10-year survival rate for pediatric ependymoma is about 5070% (57 of 10) in the United States. […] Factors that affect the chance of cure include: Location of the tumor, Type and grade of the tumor, If the cancer has spread to other parts of the body, If surgery can completely remove the tumor, Age at diagnosis, If the cancer has come back after treatment, Molecular features of the tumor. […] Children with certain types of ependymoma tend to have a better chance for long-term survival. But late recurrences or relapse (beyond 5 years) may occur. […] Ependymoma is hard to cure without successful surgery to remove the tumor. If surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor. […] Prognosis for ependymoma depends on many factors. Every case is different. Always talk to your care team about your childs case. […] Your childs doctor is the best source of information about prognosis.
- #41 Ependymoma in Children and Teens – Together by St. Judeâ¢https://together.stjude.org/en-us/conditions/cancers/ependymoma.html
The overall 10-year survival rate for pediatric ependymoma is about 5070% (57 of 10) in the United States. […] Factors that affect the chance of cure include: Location of the tumor, Type and grade of the tumor, If the cancer has spread to other parts of the body, If surgery can completely remove the tumor, Age at diagnosis, If the cancer has come back after treatment, Molecular features of the tumor. […] Children with certain types of ependymoma tend to have a better chance for long-term survival. But late recurrences or relapse (beyond 5 years) may occur. […] Ependymoma is hard to cure without successful surgery to remove the tumor. If surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor. […] Prognosis for ependymoma depends on many factors. Every case is different. Always talk to your care team about your childs case. […] Your childs doctor is the best source of information about prognosis.
- #42 Ependymoma: Symptoms, Treatment, Prognosis & Typeshttps://my.clevelandclinic.org/health/diseases/23147-ependymoma
The five-year survival rate for ependymomas is nearly 85%. That means around 85% of people are still alive within five years of being diagnosed. But this number may not apply to you or your unique situation. Your providers will tell you what can expect. […] Theres no cure for cancer, but its possible to remove or destroy a grade 3 ependymoma with treatment. […] Remember, theres always a chance an ependymoma regrows (recurs), even after youve been declared cancer-free. Thats much more likely with grade 3 tumors. Your providers and surgeon will tell you what to expect based on your age and overall health.
- #43 Ependymoma in Children and Teens – Together by St. Judeâ¢https://together.stjude.org/en-us/conditions/cancers/ependymoma.html
The overall 10-year survival rate for pediatric ependymoma is about 5070% (57 of 10) in the United States. […] Factors that affect the chance of cure include: Location of the tumor, Type and grade of the tumor, If the cancer has spread to other parts of the body, If surgery can completely remove the tumor, Age at diagnosis, If the cancer has come back after treatment, Molecular features of the tumor. […] Children with certain types of ependymoma tend to have a better chance for long-term survival. But late recurrences or relapse (beyond 5 years) may occur. […] Ependymoma is hard to cure without successful surgery to remove the tumor. If surgery is incomplete or ependymoma recurs, the long-term prognosis is usually poor. […] Prognosis for ependymoma depends on many factors. Every case is different. Always talk to your care team about your childs case. […] Your childs doctor is the best source of information about prognosis.