Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
Sign up to receive an email alert when a new Case of the Week is posted.
October 26, 2009
NF-1, Extensive Plexiform Neurofibromas
- Plexiform neurofibroma is a tumor of the peripheral nerves which is common in neurofibromatosis type 1. It presents as diffuse and elongated swelling along the course of the nerves and tends to infiltrate deeper structures like fascia, muscles and bone. It is commonly seen in connection with the branches of trigeminal nerve.
- They appear in utero or during early childhood in some patients, therefore, they interact with the skull, orbital structures, and globes while they are still developing & can deform them.
- Tumors affect nearby bone and invade almost all orbital structures, including: globe, extraocular muscles, optic nerve sheath, and branches of sensory nerves.
- The risk of degeneration to neurofibrosarcoma is 2-12%.
Current Issue
Case Collections
