plexiform neurofibroma

A large plexiform neurofibroma in the leg of a 6-year old male.

As of 2002, the primary treatment option for plexiform neurofibroma was surgery.

Plexiform neurofibroma are more difficult to treat and can transform into malignant tumors.

Plexiform neurofibroma can cause pain, disfigurement, neurological and other clinical deficits.

Once a plexiform neurofibroma has undergone malignant transformation, radiation and chemotherapy can be used as treatment.

A plexiform neurofibroma has a lifetime risk of 8-12% of transformation into a malignant tumor.

When a plexiform neurofibroma manifests on a leg or arm, it will cause extra blood circulation, and may thus accelerate the growth of the limb.

Sorafenib is being studied for treatment of unresectable plexiform neurofibroma and low-grade astrocytomas.

Plexiform neurofibroma: Often congenital.

Cancer can arise in the form of Malignant peripheral nerve sheath tumor resulting from malignant degeneration of a plexiform neurofibroma.

Two or more neurofibromas on or under the skin, or one plexiform neurofibroma (a large cluster of tumors involving multiple nerves); neurofibromas are the subcutaneous bumps characteristic of the disease, and increase in number with age.