About Juvenile Pilocytic Astrocytoma
Juvenile pilocytic astrocytoma (JPA) is the name for a rare brain tumor that occurs in childhood. It is also sometimes called, more simply, pilocytic astrocytoma. The tumor is found to be benign most of the time. In most cases, the tumor grows slowly and does not spread. This means the tumor is non-infiltrating and is classed as a Grade I astrocytoma or WHO grade I glioma.
According to WebMD, two percent of all brain tumors in teens and children are pilocytic astrocytomas. A report from the National Institutes of Health states that the incidence of pilocytic astrocytomas in patients aged 19 and younger is 0.87 for each 100,000.
This type of tumor can become large and is located most frequently in the optic nerve pathway, cerebrum, cerebellum or in the brain stem.
Star-shaped brain cells known as astrocytes work with other cells to create the tissue that protects and surrounds nerve cells within the spinal cord and brain. JPA can develop from these astrocytes.
The exact symptoms patients with JPA will experience are dependent upon the location of the tumor. The symptoms that may occur with JPA include:
- Vision problems
- Balance problems
Though rare, seizures do occur in some patients. Other symptoms that are seen less often in patients with JPA include:
- Language problems
- Mood change
- Personality change
- Memory loss
The National Institutes of Health reports that about 15 percent of all pilocytic astrocytoma patients have an “inherited tumor predisposition syndrome neurofibromatosis type 1 (NF1).” Aside from that 15 percent, patients usually do not have astrocytomas in their family history.
Some of the tests that may be ordered to make a diagnosis of JPA may be an MRI, a CT scan or both. An IV-administered contrast agent will be used to help identify the tumor among the surrounding brain tissue.
Because the tumor is usually benign, sometimes no treatment is used at all. If the tumor changes, a treatment of surgical removal or radiation may be used. If surgical removal is used, radiation treatment is usually only recommended in cases in which the complete tumor was not removed.
Another option is called stereotactic radiosurgery. This process uses a very focused radiation beam to target the specific area that is affected, leaving the surrounding area untouched. Chemotherapy is another option that may be used in the treatment of JPA.
Due to the nature of the juvenile pilocytic astrocytoma, patients usually have an excellent prognosis. The slow growth and lack of spreading is responsible for this.
Juvenile Pilocytic Astrocytoma. Children’s Health. WebMD. Last updated February 21, 2010. https://children.webmd.com/juvenile-pilocytic-astrocytoma
Astrocytoma. WebMD. Last updated February 22, 2010. https://www.webmd.com/cancer/brain-cancer/astrocytoma
Juvenile Pilocytic Astrocytomas. Columbia University Medical Center: Department of Neurological Surgery. https://www.columbianeurosurgery.org/conditions/juvenile-pilocytic-astrocytomas/
Gene Expression Profiling of NF-1 Associated and Sporadic Pilocytic Astrocytoma Identifies Aldehyde Dehydrogenase 1 Family, Member L1 (ALDH1L1) as an Underexpressed Candidate Biomarker in Aggressive Subtypes. Fausto J. Rodriguez, MD; Caterina Giannini, MD; Yan W. Asmann, PhD; Mukesh K. Sharma, PhD; Arie Perry, MD; Kathleen M. Tibbetts, BS; Robert B. Jenkins, MD, PhD; Bernd W. Scheithauer, MD; Shrikant Anant, PhD; Sarah Jenkins, MS; Charles G. Ebehart, MD, PhD; Jann N. Sarkaria, MD; and David H. Gutmann, MD, PhD. J Neuropathol Exp Neurol 2008 December, 67(12): 1194-1204. National Institutes of Health. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2730602/