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2006 Series : May 9, 2006

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A  25-year-old male presents with decreased vision in the left eye and is noted to have the above skin and retinal findings. You suspect that the patient has a retinal astrocytic hamartoma and this disease represents one of the phakomatoses.

Which one of the following is true regarding this disease?   

a. autosomal dominant inheritance
b. Manifestations of the disease are limited only to the eye and skin.
c. The retinal lesions are malignant and the eye must be immediately enucleated to prevent metastases to the central nervous system.
d.  The skin lesions are only located on the face and may fade with time.

For answers to the above, click here on or after May 16, 2006.