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 nerveso Adults o Single , smooth, firmo 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 visionErodes into bone, orbit & deeper

structuresMyxomatous degeneration

Generalized von Recklinghausen’s disease 1 in 4000 births Inherited autosomal disease

2 types-Type 1- chr.17 mutationType 2- chr.22 mutation

Soft, non-tender, multiple

Skeletal deformitiesNeurological disturbance

Café au lait spotsLisch nodulesMay be associated with MEN type2b

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

Pachydermatocele

Variant of plexiform NFNeck 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

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