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Neurofibroma
Definition

 Benign tumor arising from CT of nerve
  sheath.
 Horizontal mobility only.
 Consists of neural (ectodermal) &
  fibrous(mesodermal) elements.
 Benign
 Single/ multiple
 Fusiform swelling in the direction of nerve
  fibre.
Sites
 Cranial
 Spinal
 peripheral
Types
   Nodular
   Plexiform
   Generalised
   Elephantiatic
   Pachy dermatocoele
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.
Plexiform
 Along the distribution of 5th cranial nerve-
  ophthalmic division
 Enormous size
 Skin thickening
 Hangs downwards
 Cosmetic problem
 Variant- patchy dermatocoele
 Tingling paresthesia
 Obstructs vision
 Erodes into bone, orbit & deeper
  structures
 Myxomatous degeneration
Neuro fibroma
Generalized
  von Recklinghausen’s disease
 1 in 4000 births
 Inherited autosomal disease


    2 types-
       Type 1- chr.17 mutation
       Type 2- chr.22 mutation
   Soft, non-tender, multiple



    Café au lait spots
    Lisch nodules
    May be associated with MEN type2b

 Skeletal deformities
 Neurological disturbance
Neuro fibroma
Neuro fibroma
Elephantiatic
 Advanced   stage of plexiform variety
 Involves limbs
 Dry, coarse, thick skin
 Congenital origin
Pachydermato
    cele
 Variant of plexiform NF
 Neck is involved
 Familial type- assoc. with scoliosis
 Intestinal NF- ppts. intussusception
Complications
 Sarcomatous changes
 Cystic degeneration
 Haemorrhage into tissues
 Neurological deficits
 Muscle atrophy
 Spinal dumbell tumor
 Kyphoscoliosis osteoporosis
Treatment
Excision-
 Symptomatic
 Cosmetic
 Recent increase in size
 Malignant transformation
Thank you…

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Neuro fibroma

  • 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.
  • 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