Date post: | 11-May-2015 |
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Health & Medicine |
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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 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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