Juvenile Pilocytic Astrocytoma (JPA)

Overview


Tumour Group: Gliomas and Pediatric Brain Tumours
WHO Grade: Grade I
Prevalence/Incidence: Pilocytic astrocytomas comprise approximately 5-6% of all gliomas. This tumour type has an overall incidence of 0.37 per 100,000 persons per year.
Typical Age Range: Pilocytic astrocytomas are typically seen in children and young adults. They can occur at all ages in children and teens, but the median age for a juvenile pilocytic astrocytoma (JPA) is 5 to 14 years. This tumour type is unusual in infants and very rare in adults.

Contents
Description of Tumour
Symptoms
Treatment / Standard of Care
Prognosis
References

Description of Tumour


JPAs are also known as pilocytic astrocytomas. These tumours grow in the cerebrum, optic nerve pathways, brainstem and cerebellum. They are slow-growing and are the most common subtype of a low-grade glioma.

  • In children they are very common in the cerebellum and may lead to raised intracranial pressure.
  • Pathologically they have relatively well-defined borders and Rosenthal fibers are seen under the microscope.
  • These types of astrocytomas typically stay in the area where they started and do not spread. They are considered the most non-malignant of all the astrocytomas.

Symptoms


Symptoms depend on the size and location of the tumour and may include:

  • Changes in behaviour
  • Headaches and vomiting (due to raised intracranial pressure)
  • Lack of coordination and loss of balance (due to cerebellar involvement)
  • Memory loss
  • Seizures

Treatment / Standard of Care


  • Surgery is usually the most important treatment type for JPA.Complete surgical removal is generally possible for JPAs arising in the cerebellum and then no further treatment is necessary.
  • If total removal is not possible, chemotherapy is often used to shrink these tumours. Sometimes then surgery is possible and sometimes the tumour will not progress further after chemotherapy.
  • Occasionally radiation therapy is necessary (for example if the tumour has grown into the brainstem).

The role of chemotherapy is changing in JPA treatment. Some response has now been found with chemotherapeutic agents.

Prognosis  


A prognosis is an estimate of the likely progress of a disease after a diagnosis, based on an average patient group. Since every person is different, please take time to talk with your health care team about how this information applies to you.

By clicking on 'Expand,' a statistic on the prognosis for juvenile pilocytic astrocytomas will be shown.

Expand for Prognosis Information

For brain tumour patients, a prognosis depends on several factors, which can include age and other health issues, the size of the tumour, its molecular profile, the type of tumour, how much can be removed and its response to treatments such as chemotherapy and radiation therapy.

For low-grade astrocytomas, like JPA, the 10-year overall survival was 83% in a large retrospective series. There was improved survival with:

  • Pilocytic astrocytoma
  • Hemispheric tumour location
  • Greater extent of resection

References


 Astrocytomas. The National Brain Tumour Society, n.d. Web. July 9, 2013.

 Juvenile pilocytic astrocytoma " American Brain Tumour Association. American Brain Tumour Association, n.d. Web. July 9, 2013.

Louis, David N. WHO Classification of Tumours of the Central Nervous System. Lyon: International Agency for Research on Cancer, 2007. Print.

"National Cancer Institute." Childhood Astrocytomas Treatment (PDQ®) -. N.p., n.d. Web. 21 Sept. 2013.

Pediatric Brain Tumour Handbook 6th Edition. Brain Tumour Foundation of Canada. 2012.

"Result Filters." National Center for Biotechnology Information. U.S. National Library of Medicine, n.d. Web. 21 Sept. 2013.

Image credited to Hellerhoff (Own work) [CC-BY-SA-3.0 (http://creativecommons.org/licenses/by-sa/3.0)], via Wikimedia Commons

 

You can also download this information as a Information Sheet on Juvenile Pilocytic Astrocytoma (pdf).

 

For Nik, the brain tumour journey has been very challenging. At 22, Nik received the shocking news that he had a large mass in his right occipital lobe. While the process leading to his diagnosis was slow, his surgery was scheduled almost immediately. Following a second surgery, Nik was re-diagnosed with a different tumour type, a pilocytic astrocytoma. While this new diagnosis was scary, Nik focused on remaining positive. Read Nik's uplifting story >>

 

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