• Tectal Glioma

    A tectal glioma is a low-grade, slow-growing brain tumor in the tectum, the roof of the brain stem. The brain stem controls vital body functions such as breathing, heart rate and blood pressure.

    Despite their origin in a critical part of the body, tectal gliomas have a very high cure rate and the long-term prognosis is usually excellent. Most children with tectal gliomas develop these brain tumors between the ages of 3 and 16. Tectal gliomas tend to develop spontaneously, which means that there is no known environmental or genetic factor that doctors suspect could have caused the tumor to grow.

    Low-grade gliomas are a family of brain tumors that are typically non malignant and rarely aggressive.The tumors originate in glial cells, which support and nourish neurons in the brain.

    Tectal Glioma Treatment at Dana-Farber/Boston Children's

    Children with tectal gliomas are treated at Dana-Farber/Boston Children's through our Glioma Program – one of the world’s largest pediatric glioma treatment programs. Our internationally-recognized pediatric brain tumor specialists have extensive expertise in treating all types of gliomas, including tectal gliomas. Our glioma specialists – a team of neuro-oncologists, surgeons, pathologists and radiation oncologists – focus solely on the care of children diagnosed with gliomas. Our program also offers families the chance to have their child's tumor molecularly profiled (as long as a biopsy can be taken), which may help identify opportunities for targeted treatment (precision medicine).

    How are tectal gliomas classified?

    All gliomas, including tectal gliomas, are graded using a scale developed by The World Health Organization (WHO) according to their appearance under a microscope and several other factors. These grades are useful to doctors as they predict how the tumor will progress. The WHO grades are:

    • grade I (benign)
    • grade II (atypical)
    • grade III (anaplastic, which references a lack of structure in the cell)
    • grade IV (sarcomatous, the most serious kind of tumor)

    Tectal gliomas are classified as grade I or grade II tumors – also known as low-grade gliomas – and are generally not malignant.

    What are the causes and symptoms of tectal gliomas?

    Tectal gliomas occur spontaneously, which means that there is no known environmental or genetic reason for your child's condition. Scientists know that these types of tumors emerge in the brain stem's glial cells, which make connective tissue in the brain. The exact cause of this abnormal growth isn’t fully understood.

    It’s important to understand that there’s nothing you could have done – or avoided doing – that would have prevented your child’s tumor from developing.

    The symptoms of a tectal glioma can be non-specific, and might mimic those of other, more common ailments. Since these tumors grow relatively slowly, your child may see the doctor with problems that have been occurring for many months. Or, symptoms may come on rapidly. While tectal glioma symptoms vary from child to child, the most common include:

    • Indications of increased pressure in the brain, including headache (usually upon awakening in the morning), vomiting and fatigue
    • Abnormal eye movements
    • Difficulty walking

    Because many of these symptoms can also point to other conditions, it’s important to have your child evaluated by a qualified medical professional right away for an accurate diagnosis and prompt initiation of treatment.

    How are tectal gliomas diagnosed?

    The first step in treating your child is forming an accurate and complete diagnosis. Tectal gliomas are typically diagnosed using a combination of imaging and biopsy. Your child’s physician may order a number of different tests including:

    • A physical exam and complete medical history.
    • A neurological exam to assess reflexes, muscle strength, eye and mouth movement, coordination and alertness.
    • Magnetic resonance imaging, a diagnostic procedure that produces detailed images. An MRI uses a combination of large magnets, radiofrequencies and a computer to analyze organs and structures within the body. For tectal gliomas, MRIs of the brain and spine are usually done.
    • Computerized tomography scan (also called a CT or CAT scan), an imaging technique that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of your child’s body. CT scans are more detailed than general x-rays. For tectal gliomas, a CT scan of the brain is usually done.
    • Biopsy, a tissue sample from the tumor taken through a needle during a simple surgical procedure.

    There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, we will meet with you and your family to discuss the results and outline the best possible treatment options.

    What are the treatments for tectal gliomas?

    Treatment of tectal gliomas generally focuses on relieving symptoms caused by increased pressure within the brain and monitoring the tumor for growth or complications. Relieving cranial pressure is usually all a patient needs for a good prognosis. Chemotherapy is usually used as treatment for those children who require further treatment is required. Radiation therapy is usually avoided for the majority of children with tectal gliomas.

    Our experts address intracranial pressure by:

    • Surgically inserting a device called a shunt, which drains excess fluid from your child's body.
    • Performing a procedure called a endoscopic third ventriculostomy, an alternative to a shunt in which the surgeon creates a pathway to drain fluid into the brain's third ventricle.
    • The oral steroid dexamethasone may also be given to relieve pressure.

    As a follow-up, your child will be followed for evidence of progressive disease through frequent MRI scans (about every three months to start). Spinal fluid diversion has a high cure rate and is sufficient therapy for the majority of patients.

    What is the latest research on tectal glioma?

    Research is a top priority at Dana-Farber/Boston Children's, and our physicians work continuously to translate laboratory findings into clinical therapies and find ways to improve survival while reducing the toxicity and long-term impact of treatment. For instance, a 2014 study led by Peter Manley, MD, documented the excellent long-term survival among patients with low-grade gliomas, and the negative impact of radiation therapy – long a mainstay of pediatric brain tumor treatment – on that survival.

    Dana-Farber/Boston Children's houses the Pediatric Low-Grade Astrocytoma (PLGA) Program, the world's only multidisciplinary clinical and research program dedicated to pediatric low-grade gliomas. Established in 2007 with support from the PLGA Foundation, the program takes a multifaceted approach to finding more effective, less toxic treatments and a cure for children battling brain tumors, and has become the standard bearer for the research and care of pediatric brain tumors. Our pediatric neuro-oncologists, including Mark Kieran, MD, PhD, clinical director of the Brain Tumor Center at Dana-Farber/Boston Children's, and Pratiti (Mimi) Bandopadhayay, MBBS, PhD, are actively contributing to these efforts. Our program has contributed to international research efforts that have identified genomic drivers that contribute to growth of low-grade glioma. Specifically, we have identified genes that are commonly mutated in low-grade glioma. These findings are guiding clinical trials examining the activity of new drugs specifically for children with low-grade glioma.

    Clinical trials

    Clinical trials, or research studies evaluating new treatment approaches, are a major offering at Dana-Farber/Boston Children’s. For many children with rare or hard-to-treat conditions, clinical trials provide new options.

    It’s possible that your child will be eligible to participate in one of the Glioma Program’s current brain tumor clinical trials. In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG) and the Pacific Pediatric Neuro-Oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's Oncology Group. If your child has progressive or recurrent tumor, she may be eligible for a number of experimental therapies available through these groups, or from one of our independent clinical investigators.

    What is the long-term outlook for tectal glioma?

    Tectal gliomas are associated with a high cure rate. Over time, many children will demonstrate an increase in tumor size; however, most will remain asymptomatic and not require further treatment other than ongoing MRI scans.

    However, it’s important to understand that your child’s short-term health and long-term health can vary greatly depending on his specific circumstances. Prompt medical attention is very important, as is follow-up care. Follow up care is provided by our brain tumor survivorship clinic.