Neuroblastoma is a malignant tumour of neural crest cells. These neural crest cells are the precursor cells which give rise to the sympathetic nervous system. Neuroblastoma is a type of cancer that starts in the precursor, immature nerve cells seen in an embryo or fetus. Simply put, the term neuro refers to nerves, whilst blastoma means a cancer that involves immature or developing cells. It is basically a childhood tumour occurring in the very young age group,that is mostly 5 years and less. They account for 8 to 10% of childhood cancers.
Where does neuroblastoma occur ?
As it arises in the sympathetic nervous system, it can occur at any site where it is found. However, it occurs most commonly in the abdominal area.
What are the causes of neuroblastoma ?
Most neuroblastomas are sporadic cancers. In rare cases, familial neuroblastoma seems to occur because of gene changes inherited from a parent. Inherited changes in the ALK oncogene are seen in most of the hereditary neuroblastoma. Also, some cases of neuroblastoma become more aggressive or grow faster if certain genes are mutated (MYCN gene). Researchers have found some of the gene changes that may lead to neuroblastoma, but it’s still unclear what brings about these gene changes. Some gene changes of course are inherited. Some might have unknown environmental causes, but others may just be randomly occurring events.The potential role of environmental exposures remains unknown, so there is not much that can be done to prevent these cancers.
What are the clinical features of neuroblastoma ?
At the time of diagnosis, the tumour may have a wide-ranging presentation. It can be limited to a single organ (that is localized), or locally or regionally invasive, or extensively spread. Some grow rapidly and some grow slowly. Bone, bone marrow, liver and skin are amongst the most common metastatic sites. Clinically, the neuroblastoma is known for its variable behaviour. The behaviour of the neuroblastoma depends on the site at which it occurs. A majority of them occur localized in the abdomen. It may be felt as a palpable mass in the abdomen associated with digestive problems. Thoracic neuroblastomas often produce respiratory problems like respiratory distress and dysphagia.
Similarly, clinical symptoms in metastatic disease also vary widely.
How is neuroblastoma diagnosed ?
There are two tests which point to the diagnosis of neuroblastomas.
- If there are increased catecholamine metabolites in the urine- the basic catecholamines include dopamine, epinephrine (adrenaline), and norepinephrine. These hormones are converted to their metabolites, such as homovanillic acid and vanillylmandelic acid and then secreted in the urine. These can be measured and checked for, in the urine.
- If meta-iodobenzylguanidine (MIBG) scintigraphy shows positivity- it is a radiopharmaceutical used to identify and localize adrenergic tissues, such as that seen in neuroblastomas.
CT scan and /or MRI should be carried out to provide the exact anatomical information of the tumour such as its extent and measurements.
A histological study of the biopsy, under the microscope is required to confirm diagnosis. The picture of a neuroblastoma is non-specific; a neuroblastoma is a round cell tumour with very uniform, small cells seen, containing dense hyperchromatic nuclei (a common feature seen in rapidly dividing cells) and very little cytoplasm.
How are neuroblastomas treated ?
Firstly, the tumours have to be staged. This has to be done both locally and systemically. There are different treatments required at different stages of disease. Moreover, age at the time of occurrence of neuroblastoma and prognostic factors both influence treatment strategies.
Secondly, patients should be treated according to national or international treatment protocols.
The different regimens include the following.
- Surgery
- Chemotherapy
- Chemotherapy followed by surgery
- High-risk disease- high dose chemotherapy, surgery, autologous stem cell transplant, radiotherapy, immunotherapy and maturation therapy.
Briefly, localized tumours are treated by primary surgery if possible. Chemotherapy also would be needed if there are unfavourable prognostic factors in the case. Local radiotherapy may be indicated in aggressive or fast spreading tumours.
Metastatic neuroblastoma requires neoadjuvant chemotherapy followed by surgery of the primary tumour, if possible.
How does a neuroblastoma behave ?
Most localized tumours have an exceptional prognosis if removed by surgical resection. Infants less than 1 year have a better prognosis than older children regardless of tumour stage. Some of these tumours have been known to show spontaneous regression, as the child grows. On the contrary, about 60% of older children (> 1 year) with neuroblastoma show disseminated, metastatic disease at diagnosis. They have poorer outcome, in spite of intensive treatment protocols.
What is the care to be taken after the removal of a neuroblastoma ?
Metastatic diagnostic workup and monitoring post-treatment for aggressive or disseminated neuroblastomas includes imaging and scanning of the limbs, thorax, chest, retroperitoneum, and abdomen with radiography, MRI or CT scans.
How can neuroblastomas be prevented ?
Some studies suggest that having mothers take prenatal multi-vitamins or folic acid might decrease the risk of neuroblastoma. However, more research is needed to substantiate this.
If there is a family history of neuroblastoma, consult a genetic counsellor about the children’s risks of having the disease.
How to find and reach cancer specialists for neuroblastoma treatment ?
Now you can find and reach cancer specialists for neuroblastoma treatment from different cancer hospitals and destinations on a single platform, Hinfoways. You can avail opinions and information from multiple cancer specialists, cost estimates for neuroblastoma treatment from different cancer hospitals, compare things and then choose a cancer specialist or a cancer hospital for neuroblastoma treatment.
Find, reach and choose a cancer specialist for neuroblastoma treatment on Hinfoways. Make an informed choice.
Disclaimer: The content provided here is meant for general informational purposes only and hence SHOULD NOT be relied upon as a substitute for sound professional medical advice, care or evaluation by a qualified doctor/physician or other relevantly qualified healthcare provider.
