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Astrocytoma / Glioma

This family of tumors includes: Anaplastic astrocytoma, fibrillary astrocytoma, glioblastoma multiforme (GBM), juvenile pilocytic astrocytoma, pilocytic astrocytoma (PA), pilomyxoid astrocytoma (PMA), pleomorphic xanthoastrocytoma (PXA)

What is Astrocytoma / Glioma?

Astrocytomas and gliomas are a type of tumor of the brain and spinal cord that grow from cells called astrocytes. Astrocytes are a type of glial cell which make up the brain's supportive tissue. Both “astrocytoma” and “glioma” are used to refer to this type of cancer.

Astrocytomas are the most common type of childhood brain tumor. They account for about half of all central nervous system (CNS) tumors in children.

Treatment for most astrocytomas usually includes surgery to remove the tumor. Chemotherapy or radiation therapy are often used along with surgery to kill any remaining cancer cells.

Places for astrocytomas to develop include the:

  • Cerebellum
  • Cerebrum
  • Brainstem
  • Hypothalamus
  • Visual Pathway
  • Spinal cord
 

Signs and Symptoms of Astrocytoma / Glioma

Symptoms of childhood astrocytoma vary depending on the location. Some tumors do not cause any symptoms until they grow to be very large. In some cases, symptoms may be gradual and hard to notice, especially in low-grade astrocytomas. Other times, symptoms may be severe and develop quickly, especially in the case of high-grade astrocytomas.

Signs and symptoms depend on several factors including:

  • Child’s age and stage of development
  • Size of the tumor
  • Location of the tumor in the brain
  • Rate of tumor growth

Astrocytoma symptoms in children may include:

  • Headache, often worse in the morning and/or improves after vomiting
  • Nausea and vomiting
  • Unexplained weight change (gain or loss)
  • Vision problems
  • Changes in speech 
  • Changes in hearing
  • Loss of balance or problems walking
  • Fatigue or sleepiness
  • Weakness, numbness, tingling, or changes in sensation on one side (arm or leg or both) of the body
  • Seizures
  • Changes in personality or behavior
  • Changes in school performance
  • Symptoms due to changes in endocrine function such as unexplained thirst or early puberty
  • Increased head size in infants
  • Increased fullness of the fontanel ("soft spot" at the top of the skull)

Diagnosis of Astrocytoma / Glioma

Doctors test for astrocytoma in several ways. These tests include:

  • A health history and physical exam helps doctors learn about symptoms, general health, past illness, and risk factors. 
    • Risk Factors: For most children, there is no known cause of astrocytoma. However, there are certain genetic factors that may increase risk. These inherited conditions include neurofibromatosis type 1 (NF1) and tuberous sclerosis. Children with Li-Fraumeni syndrome are also more likely to have astrocytomas. Past radiation therapy to the brain also increases risk.
  • A neurological exam measures different aspects of brain function including memory, vision, hearing, muscle strength, balance, coordination, and reflexes.
  • A comprehensive eye exam evaluates different aspects of visual function including appearance of eye structures, visual acuity, visual field, and color perception. An eye exam can also detect increased pressure around the brain.
  • Imaging tests are used to help identify the tumor, see how big the tumor is, and find out what brain areas may be affected. 
    • Magnetic resonance imaging (MRI) is the main imaging technique that is usually used to evaluate astrocytoma. MRI uses radio waves and magnets to make detailed pictures of the body. The images made by MRI can give more information about the type of tumor and the potential spread of disease. An MRI is also done after surgery to see if any tumor remains.
    • Computed tomography (CT scan) uses X-rays to create cross-sectional images of the organs and tissues inside the body. The machine takes many pictures to make a very detailed image. The images are taken as a series of “slices” of the body and are saved to a computer. These slices or sections can allow very small tumors to be seen. 
  • A biopsy is usually used to diagnose astrocytoma. In a biopsy, a small sample of the tumor is removed during surgery. A pathologist looks at the tissue sample under a microscope to identify the specific type and grade of astrocytoma.
Axial MRI with size markings for an astrocytoma

Astrocytoma marked in an axial MRI

Coronal MRI with markings that show the size of an astrocytoma

Coronal MRI with size markings for an astrocytoma

Sagittal MRI with arrows pointing to astrocytomas

Sagittal MRI of astrocytoma

Grading and Staging of Astrocytoma / Glioma

Astrocytoma tumors are grouped by how they look under the microscope. The more abnormal cells look, the higher the grade.

Grade I and II tumors are considered low grade. The cells look most like normal cells and grow slowly. Low-grade tumors are less likely to cause death.

Grade III and IV tumors are considered high-grade tumors. They are aggressive and grow quickly, and these tumors are more likely to cause death.

Most astrocytomas in children are low grade, whereas astrocytomas in adults tend to be high grade.

Low-Grade Tumors
Localized
Grow slowly
Pilocytic astrocytoma (PA)
Juvenile pilocytic astrocytoma (JPA)
Pilomyxoid astrocytoma (PMA)
Fibrillary astrocytoma
Pleomorphic xanthoastrocytoma
High-Grade Tumors
Grow quickly
Spread to other places
High rate of recurrence
Anaplastic astrocytoma (AA)
Anaplastic pleomorphic xanthoastrocytoma
Glioblastoma multiforme (GBM)
Diffuse midline glioma (DIPG)

Pilocytic astrocytoma (or Juvenile Pilocytic astrocytoma) is the most common pediatric brain tumor. It is a low-grade tumor with a 90% survival rate at 10 years. It rarely progresses to a higher grade. PA is a slow growing tumor and seems to grow in phases. In children, these tumors often occur in the cerebellum and optic pathway. Infants with PA have a poorer prognosis than older children. Surgery is the main treatment for PA. However, not all tumors (such as Optic Pathway Gliomas) can be completely resected.

Prognosis for Astrocytoma / Glioma

The overall 5-year survival rate for pediatric astrocytoma is approximately 85%. Some low-grade astrocytomas have a survival rate of 95% or higher with complete resection. However, certain high-grade astrocytomas have a survival rate of 10-30%.

Factors that influence chance of cure include:

  • Type and grade of the tumor
  • If surgery can completely remove the tumor
  • Age at diagnosis
  • Whether the cancer is localized or has spread to other areas of the brain
  • Whether the child has neurofibromatosis type 1 (NF1)
  • If the cancer is new or if it has come back (recurrent)

Low-grade astrocytomas are less likely to recur. They can often be managed with surgery alone and followed with imaging. High-grade astrocytomas are difficult to treat and often recur.

Treatment of Astrocytoma / Glioma

Treatment of astroctytoma / glioma depends on the type of tumor and its location.  Tumors that are more aggressive need more intensive treatment.  Doctors will also consider the age of the patient.  Doctors try to avoid radiation therapy in very young children because of the severe side effects.

  1. When possible, surgery to remove as much of the tumor as possible is the main treatment for astryocytomas. For patients with grade I tumors, surgery alone may be an effective treatment. However, in some cases, surgery is not possible due to the location of the tumor.

  2. Chemotherapy is often used in addition to surgery or as the main treatment if surgery is not possible.

  3. Radiation therapy may be used depending on the type of tumor, location of the tumor, and the age of the child. It is often used after surgery to kill any cancer cells left behind.

  4. Targeted therapies work by acting on, or targeting, specific features of tumor cells such as genes and proteins. These drugs change molecular signals and processes to stop cancer cells from growing, dividing, or communicating. Some targeted therapies that are being studied in astrocytoma include selumetinib, trametinib, dabrafenib, and vemurafenib.

  5. Immunotherapy is a type of treatment that uses the body’s own immune system to recognize and attack cancer cells. Examples of immunotherapy drugs being studied in astrocytoma are checkpoint inhibitors. These drugs help block signals from cancer cells that tell the body’s immune cells not to attack.

Life After Pediatric Astrocytoma 

Recovery and long-term effects of astrocytoma / glioma in children depend on the type of tumor and treatments received. Periodic imaging is used to monitor patients long-term. The follow-up schedule is based on type of tumor and response to treatment. Care should include appropriate referrals to support services such as rehabilitation therapy, school services, and psychology. Steroid and anti-seizure medications may be needed to manage symptoms. An interdisciplinary team can help meet individual patient needs to promote long-term health and quality of life.

More: Life After Brain Tumors


Reviewed: June 2018