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Neuroblastoma is the most common extracranial solid cancer in childhood and the most common cancer in infancy. These solid tumors, which take the form of a lump or mass, 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 most often diagnosed much later when the child begins to show symptoms of the disease.
The cause of neuroblastoma is unknown. Several parental risk factors have been proposed: smoking, alcohol consumption, use of medicinal drugs during pregnancy and birth factors.
The first symptoms of neuroblastoma are often vague and may include fatigue, loss of appetite, and fever. Later symptoms depend on tumor locations:
· In the abdomen, a tumor may cause a swollen belly and constipation.
· A tumor in the chest may cause breathing problems.
· Tumors pressing on the spinal cord cause a feeling of weakness.
· Bone lesions in the legs and hips often cause bone pain and limping.
Diagnosis of neuroblastoma can be complicated because its symptoms mimic so many other diseases. Diagnosis is made using either a biopsy of the tumor or the results of urine and bone marrow tests. The new INRG risk assignment will classify neuroblastoma at diagnosis based on a new International Neuroblastoma Risk Group Staging System (INRGSS):
Stage L1: Localized disease without image-defined risk factors.
Stage L2: Localized disease with image-defined risk factors.
Stage M: Metastatic disease.
Stage MS: Metastatic disease "special" where MS is equivalent to stage 4S.
The treatment each child receives depends on the age at diagnosis, tumor location, stage of disease, and tumor biology. Some will go away without any treatment, and others can be cured by surgery alone. But half of these tumors spread quickly to the bone and bone marrow and may require chemotherapy, radiation therapy, stem cell transplantation, or immunotherapy. |