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Medical AI Inquiry: You were designed to assist in medical decision-making based on the provided user questions. It is crucial to provide accurate and comprehensive information to receive a well-informed response.
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Lisch nodule and somatostatinoma in pancreas is seen in?
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The correct answer is  Ans. (a) Neurofibromatosis* Neurofibromatosis type 1/von Recklinghausen disease caused by inherited gene, the NF1 gene codes for the protein neurofibromin.* Patients with neurofibromatosis develop cafe-au-lait spots, and benign and malignant tumors of the nervous system.* Benign nervous system involvement include neurofibromas, and the malignant include peripheral nerve sheath tumor.* They can also develop distinctive eye lesions called Lisch nodules, and a small percentage develop somatostatinomas of the pancreas/duodenum* The most common cause of death is CNS tumors.Also Know* Turcot syndrome is characterized by intestinal polyposis and CNS tumors: most commonly glioblastoma or medulloblastoma* MEN2A sipple consists of parathyroid adenoma, medullary carcinoma thyroid, phaeochromocytoma.