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| Neurofibroma |
| Etiology • Sporadic or associated with neurofibromatosis (von Recklinghausen's disease). • Mutation in NF1 gene whose product neurofibromin functions as tumor suppressor • Not proven whether somatic inactivation of second NF1 gene occurs in neoplasms |
| Pathogenesis • Proliferation of cells exhibiting predominantly features of Schwann cells., |
| Epidemiology • Most are solitary cutaneous or in association with peripheral nerve. • Numerous in neurofibromatosis, type 1, particularly plexiform type. |
| General Gross Description • Well circumscribed firm, gray tan dermal mass or • Irregular expansion of a peripheral nerve |
| General Microscopic Description • Proliferation of cells with delicate wavy nuclei • Stroma of plexiform variant often loose and myxoid. • Mast cells and fibroblasts mixed in • Dermal lesions have abundant collagenous stroma |
| Clinical Correlation • Benign neoplasm which may need removal for cosmetic reasons • Dermal lesions rarely become malignant • Patients with neurofibromatosis type 1 frequently develop sarcoma |
| References • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th ed. Philadelphia, W.B. Saunders, 1994, pp. pp. 1352-3. |
| Neurofibroma |
| Synopsis by: Melinda Sanders M.D. (TX0500M95400)[553] |
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