Primitive Neuroectodermal Tumors

Also known as: PNET, primitive neuroectodermal tumors.

What is primitive neuroectodermal tumors?

Primitive neuroectodermal tumors are a group of cancers (malignant) that start in nerve cells formed in the fetus during early development that haven't developed the way they should have (primitive or undifferentiated cells).  They can form in the brain/autonomic nervous system or in sites outside the brain/autonomic nervous system (peripheral PNETs), including the extremities, pelvis and the chest wall.

What causes primitive neuroectodermal tumors?

While the cause of primitive neuroectodermal tumors is unknown, they may share chromosomal abnormality/gene expression abnormalities. Certain genetic conditions increase the risk of embryonic tumors.

What are the symptoms of primitive neuroectodermal tumors?

Symptoms will depend on the site and size of the tumor and can include fatigue, vomiting, headaches, seizures or vision problems. If the tumor affects the spine, it might also cause leg pain or weakness, and bowel or bladder problems.

What are primitive neuroectodermal tumors care options?

Primitive neuroectodermal tumors are typically treated like many other cancers with surgery to remove as much of tumor as possible with or without a tube placed in a brain cavity to remove fluid (ventriculostomy). Other treatments include radiotherapy (radiation is usually not used in children less than 3 years of age because of potential long term side effects) and chemotherapy.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 7/12/2018 1:51:09 PM

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Reshma Naidoo, PhD of Nicklaus Children's Hospital is a pediatric neuropsychologist and neurorehabilitation speacialits with the Brain Institute.