Neuroblastoma natural history
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Neuroblastoma natural history On the Web |
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Risk calculators and risk factors for Neuroblastoma natural history |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Natural History
Complications
- Treatment-resistant diarrhea (vasoactive intestinal peptide secretion, among 4% of cases)
- Horner's syndrome (cervical sympathetic chain compression, among 2.4% of cases)
- Opsoclonus myoclonus syndrome and ataxia (paraneoplastic syndrome, among 1.3% of cases)
- Hypertension (catecholamine secretion or renal artery compression, among 1.3% of cases).
- Transverse myelopathy (tumor spinal cord compression, among 5% of cases)
- Anemia and decreased immunity (bone marrow metastasis)
Prognosis
The prognosis of neuroblastoma is poor and it depends on the following:
- Whether or not the tumor can be removed by surgery.
- The stage of the cancer: the size of the tumor, whether the cancer has spread
- The child’s general health
Post-treatment prognosis
After it is declared that the disease is gone, it often comes back. Further treatment is then required. This can be problematic because some treatments, such as chemotherapy, have cumulative effects and side-effects therefore can increase significantly if used again.
Intensive chemotherapy and radiation therapy have known long-term negative consequences. An estimated two of three survivors of childhood cancer will ultimately develop at least one chronic and sometimes life-threatening health problem within 20 to 30 years after the cancer diagnosis.[1][2]
Criteria
When the lesion is localized, it is generally curable. However, long-term survival for children with advanced disease is poor despite aggressive multimodality therapy.
Recent biologic and genetic characteristics have been identified, which, when added to classic clinical staging, has allowed accurate patient assignment to risk groups so that treatment strategies can be more effectively undertaken. These criteria include the age of the patient, extent of disease spread, microscopic appearance, and several other biological features, most importantly MYCN oncogene amplification, into low, intermediate, and high risk disease. The therapy for these different risk categories is very different. Low risk patients can frequently be observed without any treatment at all, while intermediate risk patients are treated with chemotherapy. High-risk neuroblastoma is treated with intensive chemotherapy, surgery, radiation therapy, bone marrow /Hematopoietic stem cell transplantation and biological-based therapy with Cys-Retinoic acid (Accutane). With current treatments, patients with low and intermediate risk disease have an excellent prognosis with cure rates above 90%. In contrast, therapy for high-risk neuroblastoma results in cures only about 30% of the time.
References
- ↑ "Childhood Cancer Survivors Face Increased Sarcoma Risk", HealthDay News, February 21, 2007
- ↑ Oeffinger et al.,"Chronic Health Conditions in Adult Survivors of Childhood Cancer", New England Journal of Medicine, October 12, 2006