This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Pathophysiology - Pancreatic Endocrine Tumors, Subject name: Surgery
Lisch nodule and somatostatinoma in pancreas is seen in? 
Select and explain. 
A. Neurofibromatosis, B. Multiple endocrine neoplasia 2A, C. Turcot syndrome, D. Familial adenomatous polyposis
A: Neurofibromatosis
Answer: A: Neurofibromatosis 
Explanation: 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.