What is neuroblastoma / wilms tumor?
A neuroblastoma or Wilms tumor is a form of kidney cancer. It is a blastoma of the kidneys, a malignant tumor that usually occurs in young children.
Etiology
A neuroblastoma arises from embryonic tissue. In most children with Wilms tumor are abnormalities on chromosome 11. It is assumed that this is activated an Wilms tumor gene and genes for which the normal kidney development can be suppressed. Despite evidence for the existence of Wilms tumor genes have been found so far only a Wilms tumor suppressor gene on chromosome 11. Abnormal nuclei indicate a malignant tumor; this is 13 percent of the cases. Syndromes associated with Wilms tumor are Beckwith-Wiedemann (visceromegalie, macro glossy, omphalocele and hyperinsulemische hypoglycemia in neonates, isolated hemihypertrophy, WAGR syndrome (Wilms tumor, aniridia, genitourethrale malformations and mental retardation), sporadic aniridia, Danys Drash (nephropathy, renal failure, male pseudohermaphroditisme and wilms tumor) and Simpson-Golabi-Behmel (overgrowth syndrome).
Epidemiology
Every year there are about 20 cases in the Netherlands. In three-quarters of cases, the tumor in children under five. There is an association with abnormalities of the urogenital system and the tumor is more common in blacks. When 5 percent of the cases, the disease in the first diagnosis present in both kidneys. In Germany, 68 percent of patients between one and five years, 16 percent are infants. The condition occurs in girls than slightly more common in boys. Of all cases of childhood cancer is 5.5 percent a Wilms tumor.
Neuroblastoma / wilms tumor symptoms
The tumor is usually noticed because the asymmetrical abdomen swells or the clothes do not fit anymore. There may be blood in the urine and some children complain of abdominal pain or a strange feeling in the stomach, but often there is hardly any pain. Also, his symptoms fever and hypertension.
Diagnostics
To diagnose an ultrasound of the abdomen is made. Because the tumor sometimes occurs on both sides, is then also in the healthy side image gebracht.Ook the tumor can spread into the vein of the kidney. There is 24-hour urine collection and a chest radiograph is made. If the image is not clear, a CT scan.
Neuroblastoma / wilms tumor treatment
The treatment consists of chemotherapy to reduce the tumor, followed by the kidney is removed and, if necessary, again following chemotherapy. Radiation therapy will be applied if the tumor has not been removed, or there is more than enough metastases are found in the lymph nodes, which occurs in only 15 percent of cases. Sometimes it is only possible to remove a portion of the kidney, depending on the size and location of the tumor.
Forecast
This type of cancer can be treated well if there are no metastases, cures on average 80 percent of patients. Also, the prognosis of metastatic disease is very good, 70 to recover 90 percent of all cases.