Plans for the development of an International Neuroblastoma Risk Group (INRG) classification system, to develop a consensus to permit comparisons of outcomes of neuroblastoma patients worldwide, are also included in the Seminar, authored by Dr John Maris, Children’s Hospital of Philadelphia, USA, and colleagues. The INRG is currently analysing data obtained from 11,054 patients treated in Europe, Japan, USA, Canada, and Australia between 1974 and 2002 so that this international classification system can be created.
Neuroblastoma is a cancer of the sympathetic nervous system - the nerve network that regulates the “fight or flight” response to the environment. When solid tumours form, which can take the form of a lump or mass, they commonly begin in one of the adrenal glands, though they can also develop in nerve tissues in the neck, chest, abdomen, or pelvis.
It is the most common solid extracranial cancer in childhood, and represents more than 7% of all childhood malignant cancers and 15% of all childhood cancer deaths. It is remarkable for its broad spectrum of clinical behaviour. Likelihood of cure varies widely according to age at diagnosis, extent of disease, and tumour biology.
The authors say: “Although substantial improvement in outcome of certain well-defined subsets of patients has been observed during the past few decades, the outcome for children with a high-risk clinical phenotype has improved only modestly, with long-term survival still less than 40%.”
There are three main sets of presenting signs and symptoms, categorised as localised tumours, metastatic disease, and 4S disease. Around 40% of patients present with localised tumours which can vary in size and occur anywhere in the sympathetic nervous system. In certain locations these tumours can cause compression of the spinal cord and resulting motor weakness in 5-15% of patients. Around half of patients present with metastatic disease, which often involves multiple tumour burdens which can make patients quite ill. This type of tumour can also metastatise bony orbits, and can lead to symptoms such as raccoon eyes (periorbital bruises). 4S disease occurs in only 5% of patients with neuroblastoma – and presents as small localised primary tumours with secondary tumours in liver, skin or bone marrow that almost always spontaneously regress.
The treatment methods used in neuroblastoma management include surgery, chemotherapy, radiotherapy, and biotherapy, as well as observation alone in carefully selected circumstances.
But new approaches to relapsed disease for patients with high-risk neuroblastoma have developed in recent years. These include cytotoxic agents, targeted delivery of radionuclides, immunotherapy, retinoids, angiogenesis inhibitors, and tyrosine kinase inhibitors.
The authors conclude that improvements in understanding of the genomic alterations associated with tumour and behaviour and patient outcome have moved the goal of precise prognosis closer.
They say: “Improved understanding of normal neurodevelopment of the sympathicoadrenal stem will help us identify the key mutational events that initiate neuroblastoma tumorigenesis. Defining these events, as well as those that reliably predict for the acquisition of a high-risk phenotype, might ultimately direct us to the key pathways that can be exploited therapeutically.”
Tony Kirby | alfa
Routing gene therapy directly into the brain
07.12.2017 | Boston Children's Hospital
New Hope for Cancer Therapies: Targeted Monitoring may help Improve Tumor Treatment
01.12.2017 | Berliner Institut für Gesundheitsforschung / Berlin Institute of Health (BIH)
MPQ scientists achieve long storage times for photonic quantum bits which break the lower bound for direct teleportation in a global quantum network.
Concerning the development of quantum memories for the realization of global quantum networks, scientists of the Quantum Dynamics Division led by Professor...
Researchers have developed a water cloaking concept based on electromagnetic forces that could eliminate an object's wake, greatly reducing its drag while...
Tiny pores at a cell's entryway act as miniature bouncers, letting in some electrically charged atoms--ions--but blocking others. Operating as exquisitely sensitive filters, these "ion channels" play a critical role in biological functions such as muscle contraction and the firing of brain cells.
To rapidly transport the right ions through the cell membrane, the tiny channels rely on a complex interplay between the ions and surrounding molecules,...
The miniaturization of the current technology of storage media is hindered by fundamental limits of quantum mechanics. A new approach consists in using so-called spin-crossover molecules as the smallest possible storage unit. Similar to normal hard drives, these special molecules can save information via their magnetic state. A research team from Kiel University has now managed to successfully place a new class of spin-crossover molecules onto a surface and to improve the molecule’s storage capacity. The storage density of conventional hard drives could therefore theoretically be increased by more than one hundred fold. The study has been published in the scientific journal Nano Letters.
Over the past few years, the building blocks of storage media have gotten ever smaller. But further miniaturization of the current technology is hindered by...
With innovative experiments, researchers at the Helmholtz-Zentrums Geesthacht and the Technical University Hamburg unravel why tiny metallic structures are extremely strong
Light-weight and simultaneously strong – porous metallic nanomaterials promise interesting applications as, for instance, for future aeroplanes with enhanced...
11.12.2017 | Event News
08.12.2017 | Event News
07.12.2017 | Event News
12.12.2017 | Physics and Astronomy
12.12.2017 | Earth Sciences
12.12.2017 | Power and Electrical Engineering