Neuro fibroma

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

7. Plexiform Along the distribution of 5 th 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

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

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

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

12. Pachydermatocele Variant of plexiform NF Neck is involved

13. Familial type- assoc. with scoliosis Intestinal NF- ppts . intussusception

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

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

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