Neurofibroma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2] Shanshan Cen, M.D. [3]

Overview

Neurofibroma may be classified into 5 subtypes: cutaneous/dermal/localized, subcutaneous, diffuse, intramuscular, and plexiform neurofibroma. Plexiform neurofibromas may be further sub-classified into diffuse and nodular plexiform.

Classification

Neurofibroma may be classified into following 5 subtypes:

Types of neurofibromas	Characteristics/Description
Cutaneous/Dermal/Localized (90%)	Most common These are skin tumors Painless Slowly growing < 5cm diameter Mostly solitary and sporadic, not associated with NF1
Subcutaneous	Common Present underneath the skin Can be single tumors or chains They are often a form of plexiform neurofibromas
Diffuse	Uncommon Squishy Poorly defined Runs through the full skin thickness Infiltrative lesions in subcutaneous fat (mostly seen in children and young adults) Edges are difficult to define Solitary lesions and not related to any inherited condition
Intramuscular	Common Usually isolated tumors in the muscle Sometimes can occur in chains and are plexiform neurofibromas
Plexiform/Intraneural	Associated with NF1 Diffuse involvement along a (large) nerve and its branches Have more connective tissue that separates the nerve fibers Gross enlargement of the nerve with nodular tumor development results in the gross pathologic appearance referred to as “bag of worms” Generally believed to be present at birth Disfiguring Affects function due to sheer size as well as neurovascular compromise

Plexiform neurofibromas can be further subclassified into following:

Types of neurofibromas	Characteristics/Description
Diffuse Plexiform	It extends through the skin into fascia and muscle Has “little fingers” that invade muscle or other tissue May have a large café au lait spot “above” it
Nodular Plexiform	Usually involves nerves coming off the spinal cord or off of larger nerves Usually enlarges or thickens the nerve Looks like little clusters of tumors along the nerve

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