Skip to main content Skip to navigation
Make an Appointment Today 1-888-884-BEAR (2327) 1-888-884-BEAR (2327)
Find a Doctor Donate
Call Us Menu
We care about your privacy. Read about your rights and how we protect your data. Get Details

Coronavirus Update:What patients and families need to know

Pediatric Schwannomatosis

Share:
Linked In Print Email

Schwannomatosis is a very rare form of neurofibromatosis that has only recently been recognized and appears to affect about 1 in 40,000 individuals. It is less well understood than NF1 and NF2, and features may vary greatly between patients.

The distinguishing feature of schwannomatosis is the development of multiple schwannomas, tumors of the peripheral nervous system that arise in the nerve sheath and that are composed of Schwann cells.

Diagnosis and treatment of schwannomatosis in children

Schwannomatosis can appear almost everywhere in the body. The dominant symptom is pain, which can be excruciatingly intense. Pain develops when a schwannoma enlarges, compresses nervesor presses on adjacent tissue. Some people experience additional neurological symptoms, such as numbness, tingling or weakness in the fingers and toes. Patients with schwannomatosis never have neurofibromas.

About one-third of patients with schwannomatosis have tumors only on a single part of the body, such as an arm, a leg or a segment of the spine. The number of schwannomas on a patient can vary widely.

There is no currently accepted medical treatment or drug for schwannomatosis, but surgical management is often effective. When tumors are completely removed pain usually subsides, although it may recur if new tumors form. When surgery is not possible, ongoing monitoring and management of pain in a multidisciplinary pain clinic is advisable.

Departments
Departments

Departments

Neurofibromatosis Institute

Learn more about our world-renowned Gilbert Family Neurofibromatosis Institute, which helps children with neurofibromatosis type 1 or 2 live more normal lives.

Invest in future cures for some of life's most devastating diseases

See other ways to give

Share your birthday with a child. Celebrate your life, and give a chance to someone who desperately wants to have as many as you.

Share your birthday with a child. Celebrate your life, and give a chance to someone who desperately wants to have as many as you. Make it happen

Maddox's Story

Maddox's family

Zhen Chao, now called Maddox, was born in China with a genetic condition called neurofibromatosis type 1 (NF1) that can cause painful or disfiguring tumors called plexiform neurofibromas. Read about Maddox.

Read More of Maddox's Story

You Might Also Be Interested In:

Roger Packer's Story

Roger J. Packer, MD, Senior Vice President, Center for Neuroscience and Behavioral Medicine, has been a key figure in the national movement in innovation and care of children with brain tumors, where he says research is “accelerating discovery” in the field. That has been magnified in the past five to seven years, he says, where there have been dramatic advances in the understanding of the biology of these tumors. Still, Dr. Packer himself is never satisfied, because children are still stricken with deadly brain tumors, and there is a need for improved treatment, with fewer side-effects.

Researchers Uncover Pediatric Brain Tumor Cells Using Precision Medicine That Could Lead to Improved Treatment

International collaborators, including researchers from Children’s National Health System, have unraveled profiles in the genetic makeup of tumor cells in children that could more readily identify their growth and response to treatment, offering the possibility of better, more precise therapy.

Roger J. Packer, MD, Named Medical Honoree from Children's Brain Tumor Foundation

Roger J. Packer, MD, was named the 2014 Medical Honoree by the Children’s Brain Tumor Foundation for his innovative work and leadership in the care of children with brain tumors.

Children’s Leader Named Key Advisor to National Brain Tumor Society Initiative

Roger Packer, MD, was named a key advisor to a National Brain Tumor Society initiative that carries out research and drug development to overcome barriers to better treatments for children with pediatric brain cancer.