Pediatric Brain and Spinal Tumors
The surgical treatment of pediatric brain and spinal cord tumors are a major focus of NYU Langone’s Division of Pediatric Neurosurgery. In cooperation with the Division of Pediatric Neuro-oncology as well as pediatric specialists in Neuropyschology, Endocrinology, Ophthalmology, Neuroradiology and Pathology, patients are evaluated and treated in a multidisciplinary framework, ensuring timely, compassionate, state-of-the-art care.
Following are common types of tumors treated by the pediatric neurosurgeons at NYU Langone:
Lumps and bumps on the skull are very common. A small number of these may enlarge to the point where they cause cosmetic problems or pain. The vast majority of these lesions are benign, such as epidermoid and dermoid tumors, cephalomhematoma, or osteomas. Some skull lesions may be local, benign tumors such as eosinophilic granulomas (EG) or bone hemangiomas. Rarely, skull tumors may be metastatic tumors that originated elsewhere in the body, such as neuroblastoma. Most of these lesions are diagnosed and treated within the first year of life.
Surgery may be indicated in these cases for diagnostic or therapeutic purposes. Usually, these operations are performed on an outpatient basis. In rare cases, large tumors may require cranioplasty (reconstruction of the skull).
Medulloblastoma or primitive neuroectodermal tumors (PNET) are the most common type of malignant brain tumors in children. They usually arise from the cerebellum (medulloblastoma), although pineal PNET and cerebral hemisphere tumors (supratentorial PNET) can occur. They may cause symptoms such as balance or gait difficulty due to compression of the nervous tissue, or symptoms such as headache, vomiting and lethargy due to obstructive hydrocephalus Supratentorial tumors may present with seizures, weakness, or raised intracranial pressure with headache, vomiting and lethargy. Complete or near-complete surgical resection of these tumors is important in order to optimize survival and prevent recurrence. After surgery, additional treatment is required, including chemotherapy and radiation.
NYU Langone’s pediatric neurosurgeons, along with our Pediatric Neuro-Oncology group, have been leaders in developing novel therapies that have been used in large multi-center trials for the treatment of these tumors. We serve as a regional and national referral site for infants with these cancerous tumors who cannot receive radiation therapy due to young age. Under the direction of Dr. Sharon Gardner from the Division of Pediatric Neuro-Oncology, NYU Langone is a world-renowned center for using high-dose chemotherapy and stem cell rescue in conjunction with aggressive surgical resection. Among the most malignant subset of these tumors, atypical teratoid/rhabdoid tumors (ATRT), we have tripled the long-term survival of infants and young children.
We have also investigated the basic biological concepts driving these cancerous tumors. Dr. Howard Weiner and his colleagues have made considerable contributions to our understanding of the molecular mechanisms involved in formation of these tumors. For a list of published articles on medulloblastoma by Dr. Weiner, click here.
MRI scans of pre- and postoperative medulloblastoma
Gliomas are the most common form of pediatric brain tumors. Unlike in adults, where these tumors are most often cancerous and inevitably fatal, pediatric gliomas are often curable with surgery or a combination of surgery and chemotherapy. Dr Wisoff has been a leader in the treatment of these tumors, including directing the largest prospective multi-institutional COG trial of surgical treatment for benign gliomas of childhood.
Cerebellar astrocytomas. The most common form of astrocytoma in children is juvenile pilocytic astrocytoma (JPA). These tumors arise in the back of the brain, the cerebellum, which is responsible for controlling balance and coordination. These tumors may present with symptoms of cerebellar dysfunction, including problems with coordination, balance, or gait. If the tumors are large, they may block the normal circulation of fluid (cerebrospinal fluid or CSF) in the brain, leading to enlargement of the ventricles (hydrocephalus). Symptoms of hydrocephalus include vomiting, headache, and lethargy. Unlike adult astrocytomas, the prognosis for children with this tumor is usually excellent. Complete surgical removal is the treatment of choice. When this is achieved, over 95% of the children will be permanently cured.
Large juvenile pilocytic astrocytoma of the cerebellum. The tumor was completely resected, and no additional treatment was required
Large hypothalamic juvenile pilocytic astrocytoma, before and after resection
Optic pathway gliomas. These tumors occur in or around the critical structures of the optic nerves and tract, the hypothalamus, and the pituitary gland. They may cause visual impairment and endocrine dysfunction, resulting in growth disturbance, precocious puberty, and developmental and cognitive problems. Such tumors are often treated with a combination of modalities, including chemotherapy, surgery, and radiotherapy or radiosurgery. Tumors that are primarily exophytic (that grow out of, rather than into, the optic system) can be radically resected with long-term control of the tumor in the majority of older children.
Brainstem gliomas. These tumors, also known as pontine glioma or diffuse infiltrating pontine glioma (DIPG), are rare and very difficult to treat. They often present with signs and symptoms of weakness and cranial nerve dysfunction, including hoarseness, facial weakness, double vision, numbness, and slurred speech. They may also cause hydrocephalus, resulting in headache, vomiting and lethargy. Treatment may include a biopsy to confirm the diagnosis, followed by chemotherapy and radiation. Some cases may be amenable to surgery, particularly those that are exophytic (that grow out of, rather than into, the brainstem). Treatment of hydrocephalus may also be required; options include endoscopic third ventriculostomy (ETV) or CSF shunting procedures.
Supratetentorial gliomas. Both superficial and deeptumors of the cerebral hemispheres cause symptoms related to their location, size and rate of growth. These may include seizures from irritation of the brain, focal neurological deficits (weakness, numbness, speech problems), headache, or hemorrhage.The prognosis for these tumors is often much better in children than in adults; benign tumors have up to a 95% chance of being permanently cured with surgical resection. Treatment typically includes surgery for diagnostic purposes and maximal tumor resection. Surgery may be facilitated by stereotactic guidance, EEG monitoring, electrophysiology and operative micro-dissection. Depending on the specific tumor type, further treatment with chemotherapy or radiation may also be required.
Low grade glioma of the left temporal lobe
NYU Langone pediatric neurosurgeons have been at the forefront of developing techniques for the treatment of tumors in deep and previous inaccessible regions of the brain including the basal ganglia, thalamus, and hypothalamus. Utilizing stereotactic navigation, a form of “GPS for the brain,” and advanced MRI imaging (diffusion tensor tractography) to map out critical brain pathways, we have been able to totally resect and cure benign tumors that previously would have received palliative radiation or chemotherapy.
Thalamic tumor: Images "A" through "D" show the tumor, "A" and "F" show motor pathways on DTI, and image "E" shows the tumor following resection
These types of tumors are rare, with approximately 300 new cases occurring annually in the U.S. Although not malignant, craniopharyngiomas may cause many problems due to their location adjacent to the pituitary gland, optic nerves and tracts, hypothalamus and third ventricle. While some tumors may be very large at the time of diagnosis, these tumors tend to be slow-growing, causing progressive visual, endocrine and cognitive problems as they get bigger. Common symptoms include headache, visual impairment (which may be very severe), growth arrest, diabetes insipidis and vomiting.
These tumors may have cystic and solid components.How best to treat these tumors is one of the most enduring controversies in pediatric neurosurgery. Total resection increases the patient’s chance of long-term disease free survival, although it may result in significant morbidity. As with many other conditions, the experience and judgment of the surgeon is critical in optimizing patient outcomes. Additional treatment options include partial resection followed by radiotherapy or radiosurgery, treatment of cystic tumors with aspiration, or intracavitary treatment with bleomycin or radioactive isotopes.
NYU Langone has a dedicated team of pediatric specialists including neuro-oncology, neuropsychology, endocrinology, ophthalmology, and social work for the management of these complex tumors. We serve a referral center for children who have failed primary treatment elsewhere as well as for young children. We have been in the vanguard of establishing programs to both cure these tumors and maintain quality of life. A 2010 editorial in the Journal of Neurosurgery: Pediatrics describes "Dr. Jeffrey Wisoff's group at New York University provides a contemporary benchmark for the management of craniopharyngioma by craniotomy."
For a list of published articles on the treatment of craniopharyngioma by Dr. Jeffrey Wisoff of NYU Langone’s Division Pediatric Neurosurgery, click here.
Sellar and supra sellar craniopharyngioma before and after surgery
Other lesions in the region of the pituitary gland that occur in children include Rathke’s cleft cyst, chordoma, pituitary adenoma and paraganglioma. Many of these tumors are treated at NYU Langone with transnasal-endoscopic techniques, avoiding the need for a craniotomy (open-skull surgery) or a traditional approach involving an incision above the gum line.
Nonfunctional pituitary adenoma
Rathke’s cleft cyst, preoperative
Rathke’s cleft cyst after endoscopic-transnasal resection
These tumors occur most commonly in the region between the pons and cerebellum, although they may also occur in the cerebral cortex.The cornerstone of treatment, as in many other pediatric brain tumors, remains maximal surgical resection. Presenting symptoms often include lower cranial nerve dysfunction, resulting in hoarseness, swallowing problems, double vision or facial weakness. Hydrocephalus may also occur. After surgery, additional treatment may be required.
Large ependymoma, in which the tumor extends into the spinal canal
After total surgical removal of the ependymoma, no tumor is seen
Although this is the most common primary tumor of the posterior fossa in adults, hemangioblastoma is uncommon in children. The case below is that of a 16-year-old boy with a family history of Von Hipple-Lindau syndrome, an inherited condition that predisposes people to development of such tumors. He presented with emesis and headache due to the tumor’s mass and hydrocephalus. The tumor was completely removed, and his symptoms disappeared.
Although these are the most common type of brain tumor in adults, metastatic tumors are relatively rare in children. Multidisciplinary approaches for such tumors are available, including surgery, chemotherapy, radiation therapy or radiosurgical treatments.
While spinal cord tumors are rare in children. a diverse group of such tumors may occur, ranging from benign and encapsulated to malignant and infiltrative. The presenting signs may include scoliosis, pain and neurological impairment, including weakness, numbness, or gait disturbance. The diagnosis is usually made by MRI, although the diagnosis may be suggested by subtle findings on plain X-rays or a CT scan.
To optimize outcomes, a variety of treatment techniques are required, including advanced intra-operative electrophysiological monitoring, micro-dissection, laser treatment, ultrasonic aspiration and others. All of these approaches are available and in regular use at NYU Langone Medical Center.
Hemangioblastomas are typically well encapsulated and very vascular spinal tumors. These tumors may occur in multiple locations, and may be familial (Von Hipple-Lindau syndrome). Complete surgical removal is considered curative.
Spinal ependymomas most commonly occur in the region of the filum terminale, a thin fibrous band extending from the end of the spinal cord. They may also occur anywhere in the spinal cord itself, arising from its central canal. Complete surgical removal may be curative, although long-term follow-up in required, since recurrences have been reported.
Large intramedullary ependymoma of the thoracic spinal cord
Astrocytomas of the spinal cord may be benign or malignant. Treatment may include a biopsy or surgical removal, guided by the surgeon’s experience and intraoperative electrophysiologic monitoring. Additional treatment with chemotherapy or radiation may also be required.