Neurofibroma is a benign tumor that arises from the connective tissue of nerves. It consists of neural and fibrous elements. Neurofibromas can be single or multiple, and form fusiform swellings along nerves. They can occur in cranial, spinal, or peripheral nerves. The main types are nodular, plexiform, generalized (von Recklinghausen's disease), elephantiatic, and pachydermatocoele. Nodular neurofibromas commonly affect peripheral nerves in adults as single, firm nodules. Plexiform neurofibromas often involve the face and can cause vision problems or bone erosion. Generalized neurofibromatosis is an inherited condition with

1. Neurofibroma

2. Definition
 Benign tumor arising from CT of nerve
sheath.
 Horizontal mobility only.

3.  Consists of neural (ectodermal) &
fibrous(mesodermal) elements.
 Benign
 Single/ multiple
 Fusiform swelling in the direction of nerve
fibre.

4. Sites
 Cranial
 Spinal
 peripheral

5. Types
 Nodular
 Plexiform
 Generalised
 Elephantiatic
 Pachy dermatocoele

6. Nodular
o Commonly affects peripheral nerves
o Adults
o Single , smooth, firm
o Moves perpendicular to the direction of the
nerve.
o Presents as painful s/c nodule.
o Skin can be lifted up.

7. Plexiform
 Along the distribution of 5th cranial nerve-
ophthalmic division
 Enormous size
 Skin thickening
 Hangs downwards
 Cosmetic problem
 Variant- patchy dermatocoele

8.  Tingling paresthesia
 Obstructs vision
 Erodes into bone, orbit & deeper
structures
 Myxomatous degeneration

10. Generalized
 von Recklinghausen’s disease
 1 in 4000 births
 Inherited autosomal disease
2 types-
Type 1- chr.17 mutation
Type 2- chr.22 mutation

11.  Soft, non-tender, multiple
Café au lait spots
Lisch nodules
May be associated with MEN type2b
 Skeletal deformities
 Neurological disturbance

14. Elephantiatic
 Advanced stage of plexiform variety
 Involves limbs
 Dry, coarse, thick skin
 Congenital origin

15. Pachydermato
cele
 Variant of plexiform NF
 Neck is involved

16.  Familial type- assoc. with scoliosis
 Intestinal NF- ppts. intussusception

17. Complications
 Sarcomatous changes
 Cystic degeneration
 Haemorrhage into tissues
 Neurological deficits
 Muscle atrophy
 Spinal dumbell tumor
 Kyphoscoliosis osteoporosis

18. Treatment
Excision-
 Symptomatic
 Cosmetic
 Recent increase in size
 Malignant transformation

19. Thank you…