Neuroblastoma is the most common type of cancer in infants. The cancer is caused as a result of embryonal malignancy of sympathetic nervous system which arises from the neuroblasts. The cancer develops in the nerve tissues of adrenal, chest or spinal cord. Neuroblastoma is listed as a “rare disease” in the U.S. by the National Institute of Health (NIH), and Orphanet in Europe. The cancer is most prevalent in infants below 5 years of age, with peak age group of 2 to 3 years.
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According to America Cancer Society, neuroblastoma accounts for over 7% of the total cancer cases in age group of 0 to 14 years of age. Statistics also suggest that the cancer equally affects both sexes across the globe. The cancer is rare and American Society of Cancer estimates over 700 cases in 2014 in the U.S. In more than 90% of the cases neuroblastoma is diagnosed before the age of 5. In rare cases, the cancer can be detected in ultrasound before birth. It also found that some forms of neuroblastoma get cured on their own, while others may require multiple treatments.
Common symptoms of abdominal neuroblastoma include abdominal pain, swelling in legs and altered bowel habits. Neuroblastoma in chest causes symptoms such as wheezing, chest pain, drooping eyelids, and unequal pupils. In majority if the cases, the cancer have already metastasized until it has diagnosed. Most common metastatic sites include bone marrow, skin, liver and bones. Less common symptoms include fever, shortness of breath, high blood pressure, watery diarrhea and uncontrolled eye movement.
Scientists suggest that neuroblastoma causes as a result of genetic mutations; however the exact cause of the genetic mutation leading to neuroblastoma is not clear. Further, only about 2% patients have a history of neuroblastoma, and siblings having history of neuroblastoma imply 10 times more chances of being diagnosed with neuroblastoma. Five year survival rate is the percentage of patients living at least 5 years after diagnosis of the cancer. In intermediate risk patients the 5 year survival rate is over 90%, however in high risk patient the rate is 40% to 50%.
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The cancer is diagnosed based on evidence of elevated level of urinary catecholamines and ultrasound or magnetic resonance imaging (MRI) scans. Biopsies are performed to confirm the diagnosis. Localized forms of neuroblastoma are generally treated by surgical removal of tumor.
Various therapeutics have been adopted to treat metastatic neuroblastoma, these include chemotherapy, immunotherapy, and targeted drug therapy. Drugs such as topotecan, irinotecan, and temozolomide, are being studied for application in neuroblastoma. It has also been found that isotretinoin reduces the risk of reoccurrence of the cancer after treatment. Xalkori (crizotinib) by Pfizer has shown promising results in treating neuroblastoma in infants. The drug targets mutations of the ALK gene which lead to development of neuroblastoma.
Other drugs approved for treatment of neuroblastoma include Adriamycin PFS (Doxorubicin Hydrochloride), Clafen (Cyclophosphamide), Vincasar PFS (Vincristine Sulfate). Various biopharmaceutical companies and research institutes are conducting research for discovery of better treatment options. A research article published in Journal of Clinical Oncology suggests that black and native American neuroblastoma patients have a higher prevalence of high risk disease. Considering restricted prevalence the global market for neuroblastoma is expected to remain small.
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