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]

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:[1][2][3]

Types of neurofibromas Characteristics/Description
Cutaneous/Dermal/Localized (90%)
  • Most common
  • Occurs as lumps or bumps on skin
  • Painless
  • Slowly growing
  • < 5cm diameter
  • Starts in teenage years or young adults and rarely starts in childhood
  • Increase in size and number over the years
  • Clearly defined borders hence, can be removed if necessary
  • Number of skin tumors in each patient varies tremendously
  • Mostly solitary and sporadic, not associated with NF1
Subcutaneous
Diffuse

(superficial)

  • Superficial
  • Uncommon
  • Feels soft and squishy
  • Usually involves scalp or trunk (often protruding out, like a “love handle”)
  • Ill defined margins, can't tell where tumor stops
  • Runs through the full skin thickness (from the surface all the way down to the base of the skin (subcutaneous fascia/fat)
  • Doesn't go deeper than fascia
  • Usually seen in early childhood and young adults
  • Usually associated with diffuse hyperpigmentation, so looks like a very large squishy café au lait spot
  • Solitary lesions and not related to any inherited condition
Intramuscular
  • Common
  • Usually isolated tumors in the muscle
  • Growths along very small nerves
  • Causes pain sometimes
  • Can be removed (leaves a scar behind)
  • Sometimes can occur as plexiform neurofibromas in form of chains or networks
Plexiform/Intraneural

(deep)

  • Deep
  • Associated with NF1
  • Diffuse involvement along a large nerve and its branches
  • Mostly internal/ intraneural
  • Can also involve small nerves and superficial skin
  • Have more connective tissue/extracellular matrix than cutaneous neurofibromas, 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 (congenital)
  • Disfiguring
  • Affects function due to sheer size as well as neurovascular compromise
  • Plexiform neurofibroma exhibits following features on T2-weighted MRI:
    • Target sign (low signal intensity centrally with a ring of high signal intensity peripherally)
    • Fascicular sign
    • May appear as a larger and more infiltrating mass with lobulated borders with inhomogeneous enhancement

Plexiform neurofibromas can be further subclassified into following:[4]

Types of neurofibromas Characteristics/Description
Diffuse Plexiform
Nodular Plexiform

References


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