Our ProgramThe USC Neurogenetics Multidisciplinary Program at Keck Medicine of USC in Los Angeles is designed to closely monitor the health of patients who have been diagnosed with certain diseases of the nervous system that occur due to family genetics or by spontaneous mutation of a gene. We offer advanced treatment to those patients if intervention becomes necessary.
As an integral component in the continuum of care for patients who were diagnosed with neurogenetic conditions as children, our program extends care to patients as they move into adulthood. We also provide care to patients who have been diagnosed as adults.
Conditions treated in the program include neurofibromatosis 1, neurofibromatosis 2, Von Hippel-Lindau syndrome, Li–Fraumeni syndrome, tuberous sclerosis, Noonan syndrome and Costello syndrome. Patients who have these conditions can be symptom free, but can also be susceptible to a variety of malignant or benign tumors, including neurofibromas, astrocytomas, papillomas, acoustic neuromas, meningiomas, many of which can affect different parts of the body.
Surgery is the preferred method of treatment for tumors arising from these conditions. If a tumor does occur, it is important that diagnosis and treatment occur quickly. Therefore, our team follows each patient on an ongoing basis. We work in concert with members of the Adolescent and Young Adult Cancer Program at USC, which offers a unique breadth and scope of multidisciplinary treatment and care for adolescents and young adults.
In cases where a tumor is in a location in the body that makes surgery impossible or inadvisable, we are able to provide radiation therapy as alternate treatment. We are offer the most advanced technologies in radiosurgery – Gamma Knife® and TrueBeam™ Stx – in one location, providing our patients with the best possible course of treatment.
Additionally, researchers at USC are dedicated to finding innovative methods of care for each individual patient as we continue to deepen our understanding of the genetics responsible for these conditions. Patients can also be considered for inclusion in clinical trials, helping to accelerate the development of new and better diagnostic and treatment tools.
ConditionsThe USC Neurogenetics Multidisciplinary Program treats patients who have developed certain conditions due to family genetics or due to a spontaneous mutation of a gene.
These conditions include:
- Neurofibromatosis 1 – an inherited disorder in which nerve tissue tumors form in the skin, bottom layer of skin, or cranial or spinal root nerves.
- Neurofibromatosis 2 – a genetic disorder in which tumors form on the nerves of the brain and spine, or the central nervous system.
- Von Hippel-Lindau syndrome – an inherited disorder characterized by tumors and cysts forming in different areas of the body.
- Li–Fraumeni syndrome – a rare, hereditary disorder that increases cancer risk in patients.
- Tuberous sclerosis – a group of two genetic disorders affecting multiple organs that causes tumors to grow.
- Noonan syndrome – an inherited disease that causes many parts of the body to develop abnormally.
- Costello syndrome – a rare disorder that affects many parts of the body and increases the risk of the formation of benign and malignant tumors.
Our expert physicians and staff understand how these conditions affect our patients’ quality of lives, and we ensure that each patient is given the individualized treatment they need. Each patient’s health is continually monitored and followed, so that if they develop a tumor as a result of these conditions, our doctors can quickly react and develop an appropriate treatment plan.
TreatmentsThe USC Neurogenetics Multidisciplinary Program works to identify patients who have certain conditions that arise as a result of family genetics, or a spontaneous mutation of a gene, and to closely monitor their health in case complications arise. In addition to identifying these conditions in adults, the program works closely with Children’s Hospital Los Angeles, so that as young patients with these conditions move into adulthood they can continue to be monitored and treated as necessary.
Patients can go years with no problems or symptoms, but some will develop tumors in different areas of their body, including neurofibromas, astrocytomas, papillomas, acoustic neuromas and meningiomas. Therefore, monitoring is essential.
Treatment is personalized to each patient’s need, whether that means surgical removal of the tumor, or radiosurgery when traditional surgery is not a good option. The program also offers patients the possibility of being placed into a clinical trial, which can provide them with innovative and developing treatments.
- Craniotomy – this procedure involves removal of a portion of the skull to allow the surgeon access to a brain tumor for removal.
- Retrosigmoid or translabyrinthine craniotomy – patients with an acoustic neuroma may be eligible for one of these procedures, in which an incision is made behind the ear to access and remove the tumor. Translabyrinthine craniotomy also involves the removal of the mastoid bone and a portion of the inner ear bone in order to access the tumor.
- Laminectomy – removal of a portion of the lamina (the vertebral bone) allows surgeons to reach and resect a spinal tumor.
- Epilepsy surgery – for patients with tuberous sclerosis who are having seizures, epilepsy surgery may be considered.
- Gamma Knife® Radiosurgery – the Gamma Knife® is not a knife, but rather is a precise way of delivering radiation to the tumor while minimizing damage to surrounding tissue in the brain. Many small beams of radiation are delivered through different parts of the brain and come together in a strong dose directly to the tumor.
- TrueBeam™ Stx – this radiosurgery system uses advanced imaging and respiration synchronization to continually adjust the shape of the radiation beam to deliver a high dose a tumor, sparing healthy tissue.
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What are patients saying about us
doctor listened attentively, which is not always how other practitioners act.Review for STEVEN L GIANNOTTA MD
Practicing LocationsKeck Hospital of USC
Keck Medicine of USC - La Cañada Flintridge
USC Healthcare Center 4
USC Norris Comprehensive Cancer Center
Practicing LocationsUSC Healthcare Center 2
Cerebrovascular Disease of the Brain and Spinal Cord, Surgical Approaches to the Cranial Base, Stereotactic Radiosurgery, Microvascular Decompression for Bell’s Palsy, Lateral Skull Base Surgery for Facial Nerve Tumors
Practicing LocationsUSC Norris Comprehensive Cancer Center
Practicing LocationsKeck Medicine of USC - Pasadena
USC Norris Comprehensive Cancer Center