Plexiform Neurofibroma: Symptoms & Signs

Medically Reviewed on 10/18/2021

A plexiform neurofibroma is a tumor the arises in the tissue that covers and protects nerves in the body outside the brain and spinal cord. Plexiform neurofibromas represent an uncommon variant of the genetic condition neurofibromatosis type 1 (NF1) in which neurofibromas arise from multiple nerves. Most plexiform neurofibromas are benign but some may become malignant.

Plexiform neurofibromas can occur in various locations throughout the body, including the face, chest, abdomen, back, neck, extremities, or internal organs. Symptoms depend on the tumor's location and size and can include

The tumors can involve connective tissue and skin folds and take on a "bag of worms" appearance.

Cause of a Plexiform Neurofibroma

A genetic mutation causes plexiform neurofibroma.

Other plexiform neurofibroma symptoms and signs


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Jameson, J. Larry, et al. Harrison's Principles of Internal Medicine, 20th Ed. New York: McGraw-Hill Education, 2018.