Plexiform Neurofibroma is also known as “Solitary Neurofibroma” or “Solitary Nerve Sheath Tumor” is a genetically inherited disorder in which nerve tissue grows to form tumor. Plexiform neurofibroma is a type of generalised neurofibromatosis, which occurs due to overgrowth of neural tissue in the subcutaneous fat.
It is an autosomal dominant which means that if one parent is suffering from it chances to get inherited is 50 %.
If Neurofibroma are small in number they can be invaginated by the fingers to form button hole test is known as “button hole test”.
Types of Neurofibromatosis
Neurofibromatosis is divided into 2 types Type 1 is peripheral types and constitute more than 70 percent of cases and Type 2 is central form.
Peripheral form includes
Lisch Nodule of Iris
Freckling of Axillary region
Cafe au lait Patches
Type 2 which is central form includes Optic nerve glioma, few cutaneous lesions, meningioma , Acoustic Neuroma so hearing loss at the age of 20 years is considered to be the hallmark for type 2 neurofibroma.
Read more about Neurofibroma from emedicine
Watch out video of same patient adult female of 25 years with plexiform neurofibroma below