The genetic factors behind different neuroblastoma subtypes and their prognoses have been studied by researchers from Charité - Universitätsmediz in Berlin.
Peripheral nervous system tumors are one of the most common types of childhood tumors. While some of these tumors, known as neuroblastoma, disappear on their own without treatment, others will continue to grow even despite intensive treatment. Their findings enable clinicians to predict the precise clinical course of the disease, and to adapt their treatment regimens accordingly. The study has been published in the prestigious journal Science*.
Neuroblastoma
Neuroblastoma is a very rare type of childhood cancer that develops in immature nerve cells [neuroblasts] of the sympathetic nervous system outside the brain.
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‘Scientists have discovered that neuroblastoma tumors only exhibit aggressive growth if these maintenance mechanisms are present.’
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One of the article's last-named authors, Prof. Dr. Johannes Schulte, of Charité's Department of Pediatrics, Division of Oncology and Hematology, sums up the study's main findings as follows: "The data produced by our work provide us with a precise means of classifying different neuroblastoma phenotypes." Working alongside pediatric oncologists from University Hospital Cologne, in addition to various other colleagues, Prof. Schulte succeeded in deciphering the genetic code of neuroblastoma cells.
The researchers identified mutations in the RAS and p53 cancer signal transduction pathways - changes that are often associated with a poor prognosis. However, the researchers also found some cases of spontaneous tumor regression in patients with these mutations. It was only by merging the genetic data on these cancer signal transduction pathways with information on the mechanisms responsible for maintaining the length of telomeres (structures found at ends of our chromosomes) that the researchers gained a better understanding of the mechanisms involved in producing different neuroblastoma subtypes. This in turn enabled them to make precise predictions regarding disease prognosis.
![Pediatric Neuroblastoma Patients Face Higher Risk of Psychological Impairment]( "Pediatric Neuroblastoma Patients Face Higher Risk of Psychological Impairment")
Pediatric neuroblastoma patients are at elevated risk for long-term psychological impairment, revealed research.
Telomeres are known to act as a 'molecular clock' in human cells. In most of the body's cells, telomeres will shorten each time the cell divides. Once a critical length is reached, the cell receives the signal to stop dividing, resulting in cell growth arrest or cell death. Stem cells and most cancer cells, however, have telomere maintenance mechanisms which ensure that telomere length remains at above the critical length, thus effectively rendering the cell immortal.
Prognosis was found to be particularly poor in patients with additional mutations affecting the above-mentioned cancer signaling pathways. Given that current treatments are ineffective in the majority of these patients, the ultimate aim is to develop personalized treatments instead of applying unspecific therapies with severe side effects that will not help the patient.
Interestingly, in patients without telomere maintenance mechanisms, mutations of the cancer signaling pathways were found to have no bearing on prognosis. Many of these patients show spontaneous tumor regression even without treatment, while others have such a good treatment response that reduced regimens with less side effects may soon become feasible.
"Our study shows that the activation of telomere maintenance mechanisms plays a crucial role in the development of malignant tumors," says Prof. Schulte. The researchers' findings also provide an opportunity to explore and develop new treatment approaches. Targeted treatments that aim to block cancer signaling pathways and telomere maintenance mechanisms constitute one such promising new avenue.
Source: Eurekalert
Pediatric Neuroblastoma Patients Face Higher Risk of Psychological Impairment
Pediatric neuroblastoma patients are at elevated risk for long-term psychological impairment, revealed research.
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Telomeres are known to act as a 'molecular clock' in human cells. In most of the body's cells, telomeres will shorten each time the cell divides. Once a critical length is reached, the cell receives the signal to stop dividing, resulting in cell growth arrest or cell death. Stem cells and most cancer cells, however, have telomere maintenance mechanisms which ensure that telomere length remains at above the critical length, thus effectively rendering the cell immortal.
Prognosis was found to be particularly poor in patients with additional mutations affecting the above-mentioned cancer signaling pathways. Given that current treatments are ineffective in the majority of these patients, the ultimate aim is to develop personalized treatments instead of applying unspecific therapies with severe side effects that will not help the patient.
Interestingly, in patients without telomere maintenance mechanisms, mutations of the cancer signaling pathways were found to have no bearing on prognosis. Many of these patients show spontaneous tumor regression even without treatment, while others have such a good treatment response that reduced regimens with less side effects may soon become feasible.
"Our study shows that the activation of telomere maintenance mechanisms plays a crucial role in the development of malignant tumors," says Prof. Schulte. The researchers' findings also provide an opportunity to explore and develop new treatment approaches. Targeted treatments that aim to block cancer signaling pathways and telomere maintenance mechanisms constitute one such promising new avenue.
Source: Eurekalert
Survivors of Neuroblastoma are at Risk of Psychological Impairment
Children with neuroblastoma, have an elevated risk of long-term psychological difficulties which may also affect performance at school, new research finds. The study also finds that these children are at risk for lower adult educational attainment.
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 Estrogen Plays a Key Role in NeuroblastomaEstrogen therapy and high levels of estrogen receptor can cause neuroblastoma cells mature into neuron-like cells that aids in developing new treatment methods. | Advertisement
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