Non-Surgical Treatment for Neurofibromatosis Type 2 Being Developed

By identifying a new group of growth factor receptors that signal to brain tumors, researchers are confident that they may discover a non-surgical treatment for Neurofibromatosis Type 2.

Neurofibromatosis Type 2 or NF2 is a disorder that causes multiple tumors to develop that affect the nervous system. These tumors are non-cancerous and are called vestibular schwannomas or acoustic neuromas.

There are two types of Neurofibromatosis; Type I and Type II. In type 1 (NF1), the tumors are called neurofibromas and can be found mainly on the skin. NF1 is often considered a skin condition but can carry complications such as learning disabilities, bone problems and nerve tumors inside the body.

NF1 is the most common of the two types affecting 1 in 2,500 people.

Despite having the same name, NF1 and NF2 are two separate conditions and have different causes and symptoms.

Neurofibromatosis Type 2 is the rarest of the two but the most dangerous. Tumors in NF2 start growing on the nerves responsible for hearing and balance. Tumors may also develop on the nerves running down the spine and on the coverings of the brain.

NF2 is caused by a misprint of a gene on Chromosome 22. This misprint can be either inherited from a parent or as a new mutation of the gene. Parents with NF2 has a 50% chance of passing on the misprinted gene to their children.

Folic Acid Reduces Risk of Kidney Cancer and Brain Tumors in Children

Folic acid is a B vitamin, specifically Vitamin B₉. Folic acid helps the body in manufacturing new cells. It is an essential vitamin. The body also produces folic acid in the form of folate.

For pregnant women, folic acid is especially important as it can prevent major birth defects of the baby's brain or spine.

Foods rich with folic acid are citrus fruits, leafy green vegetables, fruits, dried beans, peas and nuts. Enriched breads, cereals and other grain products also contain folic acid. The vitamin is also available as a dietary supplement.

Folic acid may reduce some childhood cancers

Folic acid fortification of foods may reduce the incidence of the most common type of kidney cancer and a type of brain tumors in children, finds a new study by Kimberly J. Johnson, PhD, assistant professor at the Brown School at Washington University in St. Louis, and Amy Linabery, PhD, postdoctoral fellow at the University of Minnesota.

Incidence reductions were found for Wilms' tumor, a type of kidney cancer, and primitive neuroectodermal tumors (PNET), a type of brain cancer.

Since 1998, the U.S. Food and Drug Administration has mandated fortification of foods with folic acid because earlier studies show that prenatal consumption of folic acid significantly reduces the incidence of neural tube defects in babies.

Brain Cancer Vaccine, Heat Shock Protein Peptide Complex 96 (HSPPC-96) , Against Glioblastoma Multiforme Proves Effective

A glioma is a tumor that starts in the brain or the spine. The most common place a glioma starts is the brain. Gliomas are categorized into grades. From grade I (least advanced disease — best prognosis) to grade IV (most advanced disease — worst prognosis). A grade IV glioma is called Glioblastoma multiforme and it is the most aggressive.

Unfortunately, grade 4 gliomas are the most common with human.

Glioblastoma multiforme (GBM) is a group of tumors that arises from the glia. The glia are non-neuronal cells that maintain homeostasis, form myelin, and provide support and protection for neurons in the brain_ within the central nervous system (CNS).

Patients with GBM die of their disease within 1 year and there is no long term survival of the disease. Because of this, scientists and doctors have focused a lot of time and effort in combating GBM. They have studied the disease and hopefully may find an effective cure and therapy.

Brain cancer vaccine proves effective

With help of patient groups, phase 2 clinical trial paves way for testing therapy that combines cancer vaccine with the drug Avastin

A new brain cancer vaccine called HSPPC-96 or Heat Shock Protein Peptide Complex 96, tailored to individual patients by using material from their own tumors has proven effective in a multicenter phase 2 clinical trial at extending their lives by several months or longer. The patients suffered from recurrent glioblastoma multiforme—which kills thousands of Americans every year.

Drug Makes Brain Tumors Glow Hot Pink

The hardest tumor to remove is a brain tumor. Some tumors look like part of the brain itself. To help identify the difference between tumors and healthy tissue and improve tumor resection, Doctor Andrew Sloan, Director of the Brain Tumor and Neuro-Oncology Center at UH Seidman Cancer Center, is testing an experimental drug called 5-Aminolevulinic Acid (5-ALA).

The drug makes brain tumor cells glow hot pink when illuminated with a special blue light incorporated into an operating microscope. The blue light helps to identify and remove tumor cells in a process called Fluorescent Guided Resection (FGR). This is unlike Photodynamic Therapy (PDT) where light is used to remove the tumor. FGR, on the other hand, helps the surgeon identify the tumor.

This process helps surgeons operating on the brain cancer to visualize the form of the tumor and see its edges more clearly. This allows them a more complete removal from the brain. 5-ALA is taken orally prior to surgery and also during the operation.

Compared to a person's normal tissue, high-grade gliomas (brain tumors) metabolize 5-ALA to protoporphyrin ix, a fluorescent compound that is similar in structure to that of chlorophyll found in plants. Tumors that absorb this compound glow with the blue light. Using a specially modified surgical microscope that contains the light, it enables the surgeon to see the glowing tumor tissue and help guide in removing the tumor.

"If we get out 95 to 99 percent of the tumor, we can almost essentially double the patient's survival," says Dr. Sloan.

Video: Dr. Sloan discusses 5-ALA and shows Fluorescent Guided Resection (FGR) in action.

5-Aminolevulinic Acid is not yet approved by the FDA in the United States although it is already used for FGR cancer treatment in Europe. UH Case Medical Center is one of the few hospitals studying the drug in the U.S. for brain tumor surgery.

Dr. Sloan is collaborating with David Dean, PhD, Associate Professor and Director of the Imaging Laboratory in the Department of Neurological Surgery at Case Western Reserve University. They are finding ways to improve how Fluorescent Guided Resection by precisely measuring protoporphyrin ix fluorescence in tumors using a digital fiber-optic probe during surgery. They believe that the digital probe will be both more sensitive and more precise than the current technique which is based on the surgeon's perception of how "pink" the tumor is.

"Nearly 13,000 people are diagnosed each year in the U.S. with malignant gliomas. Unfortunately, cures are rare and most patients live less than 2 years, so improved treatment options are critical," says Dr. Sloan.

About University Hospitals

University Hospitals serves the needs of patients through an integrated network of hospitals, outpatient centers and primary care physicians. At the core of our health system is University Hospitals Case Medical Center. The primary affiliate of Case Western Reserve University School of Medicine, University Hospitals Case Medical Center is home to some of the most prestigious clinical and research centers of excellence in the nation and the world, including cancer, pediatrics, women's health, orthopedics and spine, radiology and radiation oncology, neurosurgery and neuroscience, cardiology and cardiovascular surgery, organ transplantation and human genetics. Its main campus includes the internationally celebrated UH Rainbow Babies & Children's Hospital, ranked among the top children's hospitals in the nation; UH MacDonald Women's Hospital, Ohio's only hospital for women; and UH Seidman Cancer Center, part of the NCI-designated Case Comprehensive Cancer Center. For more information, go to www.uhhospitals.org