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This family of tumors includes: Anaplastic astrocytoma, fibrillary astrocytoma, glioblastoma multiforme (GBM), juvenile pilocytic astrocytoma, pilocytic astrocytoma (PA), pilomyxoid astrocytoma (PMA), pleomorphic xanthoastrocytoma (PXA)
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.
Places for astrocytomas to develop include the:
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:
Astrocytoma symptoms in children may include:
Doctors test for astrocytoma in several ways. These tests include:
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.
|Pilocytic astrocytoma (PA)
Juvenile pilocytic astrocytoma (JPA)
Pilomyxoid astrocytoma (PMA)
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.
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:
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 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.
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.
Chemotherapy is often used in addition to surgery or as the main treatment if surgery is not possible.
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.
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.
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.
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.
Reviewed: June 2018