Primitive Neuroectodermal Tumors (PNET)

Also known as: PNET, primitive neuroectodermal tumors.

What are 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: 8/27/2018 2:14:06 PM

Upcoming Events

AYA Game Night

The Adolescent and Young Adult (AYA) Program at the Nicklaus Children's Cancer Center invites oncology patients 14 years of age and older to this fun event. Food and beverages will be provided. Learn more.

Weekly Support Programs

Brain Wellness: Yoga for Kids

This program is provided by a certified yoga instructor. It offers children and teens the following benefits: managing stress through breathing, self-awareness, healthy movement and meditation. Yoga also promotes strength, flexibility, coordination and body awareness. Learn more.

From the Newsdesk

Dr. John Ragheb Contributes to Development of New CDC Guidelines for Mild Traumatic Brain Injury
Dr. John Ragheb, Director of the Division of Neurosurgery at Nicklaus Children’s Hospital, is among a group of renowned physicians who developed the first evidence-based guideline in the U.S. on mild traumatic brain injury (mTBI) and concussions among children, published by the CDC in September. 
Dr. Aaron Berger Discusses Brachial Plexus Injuries

Dr. Aaron Berger is a pediatriac hand surgeon at Nicklaus Children's Hospital. For more information about the Brachial Plexus and Peripheral Nerve Disorders Program, please visit