Definition of Sympathicoblastoma
Sympathicoblastoma: A childhood tumor, better known today as neuroblastoma, that arises in the adrenal gland or in tissue in the nervous system that is related to the adrenal gland. Neuroblastoma affects newborn babies and children up through the age of 10. In the adrenal it develops in the medulla (the central part) of the gland. In the nervous system it arises in one of the ganglia of the autonomic nervous system; a neuroblastoma so arising is sometimes termed a sympathicoblastoma.
Neuroblastoma is the most common solid tumor outside the brain in infants and children. There are about 600 new cases diagnosed each year in the US. This tumor can regress (go away) on its own. Unfortunately, most children over a age of year have metastatic disease, disease that has already spread -- the spread is most commonly to the lymph nodes, liver, lungs, bones, and bone marrow -- by the time of diagnosis, so there is often a very poor outcome despite aggressive therapy.
Neuroblastoma is predominantly a tumor of early childhood; two thirds of children with neuroblastoma are diagnosed when they are younger than 5 years of age. It is often present at birth but usually is not detected until later; in rare cases, neuroblastoma can be detected before birth by fetal ultrasound.
The most common symptoms of neuroblastoma are the result of pressure by the tumor or bone pain from cancer that has spread to the bone. Protruding eyes and dark circles around the eyes are common and are caused by cancer that has spread to the area behind the eye. Neuroblastomas may compress the spinal cord, causing paralysis. Fever, anemia, and high blood pressure are found occasionally. Rarely, children may have severe watery diarrhea, uncoordinated or jerky muscle movements, or uncontrollable eye movement.
Approximately 70% of all patients with neuroblastoma have metastatic disease at diagnosis. The prognosis for patients with neuroblastoma is related to their age at diagnosis (the younger, the better the prognosis), clinical stage of their disease, and (in patients older than 1 year of age) whether they do or do not have regional lymph node involvement by the tumor.
Dr. Katherine Matthay and colleagues reported in The New England Journal of Medicine that autologous bone marrow transplantation (bone marrow from the patient) and the drug 13-cis- retinoic acid, alone or in combination, significantly improved event-free in children with high-risk neuroblastoma who normally have a dismal prognosis.
In Japan a program was initiated to screen infants for neuroblastoma by testing for substances called catecholamines (made by the tumor) in their urine. However, a large population-based North American study in which most infants in Quebec were screened at ages 3 weeks and 6 months has shown that screening detects many neuroblastomas with favorable characteristics that would never have been detected clinically, apparently because the tumors would have spontaneously regressed. The screening did not decrease the morbidity (illness) or mortality (death) rate. A large study in Germany also showed that screening at one year of age does not reduce the mortality from neuroblastoma. From both studies, which appeared in The New England Journal of Medicine, it can be concluded that screening infants for neuroblastoma is not warranted.
Sources:
- Matthay KK et al. Treatment of High-Risk Neuroblastoma with Intensive Chemotherapy, Radiotherapy, Autologous Bone Marrow Transplantation, and 13-cis-Retinoic Acid. N Engl J Med 1999;341:1165- 1173.
- Woods WG et al. Screening of Infants and Mortality Due to Neuroblastoma. N Engl J Med 2002;346:1041-1046.
- Schilling FH et al. Neuroblastoma Screening at One Year of Age. N Engl J Med 2002;346:1047-1053.