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Images of Memorable Cases: Case 98

Module by: Herbert L. Fred, MD, Hendrik A. van Dijk

Case_98-pres1-1.jpg

Case_98-pres1-2.jpg

98. Neurofibromatosis Type 2

An autosomal dominant disorder affecting about one in 50,000 individuals. Diagnostic criteria for this type include bilateral cranial nerve VIII tumors or a first-degree relative with type 2, and either a unilateral cranial nerve VIII tumor or two of the following: dermal or subcutaneous neurofibromas, plexiform neurofibroma, Schwannoma, glioma, meningioma, or juvenile subcapsular lenticular opacity.

The 43-year-old woman shown had scattered dermal and subcutaneous neurofibromas together with plexiform neurofibromas of both breasts and left foot. Her mother had bilateral cranial nerve VIII tumors.

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