Brain and Spinal Cord Tumors
The Pediatric Neurosurgery Program in the Department of Neurosurgery uses the latest diagnostics and therapies to diagnose and treat benign and malignant central nervous system tumors. By combining neurosurgical expertise with stereotactic guidance and intraoperative MRI for monitoring of tumor resection, surgeons are typically able to maximize tumor removal while maintaining patient safety.
The care and treatment of children with brain and spinal cord tumors are discussed at the multidisciplinary tumor board meetings. Treatment is enhanced with access to the department's tumor vaccine program, which focuses on using the patient's own immune system in helping to control tumor growth. The department participates in National Institutes of Health-sponsored clinical trials and the National Children's Oncology Group clinical trials, enabling the medical team to provide leading-edge care for pediatric patients with spinal cord tumors.
Our team of experts in genetics, neurosurgery, orthopedics neurology, oncology, nephrology, cardiology and cardiothoracic surgery have significant experience and expertise in the treatment of children with group of conditions known collectively as neurocutaneous syndromes. These include:
- Neurofibromatosis: Neurophacomatosis is the most common form of neurocutaenous syndromes knows as neurophacomatosis. There are two types of neurofibromatosis, NF-1 and NF-2, both of which are genetic disorder affecting the nervous system. These disorders result in tumors of the nervous system and can produce other abnormalities, including bone deformities and skin changes. Most cases of neurofibromatosis arise through new mutations in an individual's genes and can be passed on to subsequent generations. Treatments for neurofibromatosis are aimed at controlling symptoms and resulting deformities. The expert team of pediatric specialists in the Pediatric Neurosurgery Program have extensive experience in the medical and surgical care of patients with neurofibromatosis.
- Tuberous sclerosis: Tuberous sclerosis is a rare multisystem genetic condition that results in the growth of benign (nonmalignant) tumors in the brain and/or the kidneys, heart, eyes, lungs and skin. The name comes from the “tuber or potatolike” nodules in the brain which calcify with age and become hard. The central nervous system is affected and can result in severe mental retardation, seizures, developmental delays, behavioral problems as well as skin abnormalities and kidney disease. Infant seizures can occur as early as the day their born. The condition is believed to affect one in 6,000 newborns, approximately 1 to 2 million people worldwide. There is no cure for tuberous sclerosis with treatment options generally addressing the individual’s symptoms, including the use of anti-epileptic drugs for seizures, medications for behavioral problems and surgery.
- Sturge-Weber syndrome: Pediatric Sturge-Weber syndrome is a neurological disorder characterized by seizures and a port-wine birthmark on the forehead and upper eyelid of one side of the face. There is a loss of nerve cells and calcification of tissue in the cerebral cortex of the brain on the same side of the body as the birthmark. Seizures and convulsions that begin in infancy may worsen with age. Other symptoms may include muscle weakness, mental retardation and developmental delays, glaucoma and an enlarged and bulging eye. The development of seizures and age of onset may correlate to the degree of neurological involvement. Treatment is often symptomatic including laser treatments for the port-wine stain, surgery for glaucoma and physical therapy. The incidence of pediatric Sturge-Weber syndrome is estimated at one for every 50,000 births. For children with debilitating surgically intractable epilepsy, surgery can be very effective in controlling or, in many cases, eliminating seizures.
- Von Hippel-Lindau (VHL): Von Hippel-Lindau disease is a rare condition in which hemangioblastomas (tumors of the central nervous system that originate from the vascular system) are found in the central nervous system, kidneys and retina. The disease is caused by mutations of the VHL tumor suppressor gene. As long as one copy of the VHL gene is present, tumors do not form. Von Hippel-Lindau disease is also considered an autosomal (a disease caused by a dominant mutant gene) disease which means that if one copy of the gene is inherited, there is a high probability that a second mutation will develop within the body. If the mutations occurs in the second copy as well, there is no functional VHL protein to protect the body. Approximately 80 percent of the mutant VHL genes are inherited with the remaining 20 percent a result of the new mutation that takes place early in fetal development. Von Hippel-Lindau disease is incurable, but the symptoms of the disease may be managed.