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What is Which of the following condition is shown below? and explain in detail?
Ans. (A) Plexiform neurofibroma(Ref: Robbins and Cotran Pathologic Basis of Disease 10th ed; pg. 326)NF-1Gene mutated is located on chromosome 17qCharacterised by cafe au lait spots, Lisch nodules, dysplasia of lesser wing of sphenoid and risk of developing meningiomaNF-2Due to mutation in gene located on chromosome 22qCharacterised by cafe au lait spots and bilateral vestibular schwannomaMarfan's SyndromeDue to defect in fibrillinCharacterised by hyperflexibility of joints, subluxation of lens and cardiac problems (mitral valve prolapse, bicuspid aortic valve)Von Hippel Lindau SyndromeGene is located on chromosome 3pHigh risk of developing clear cell renal cell carcinoma and cerebellar hemangioblastoma