Lisch nodule and somatostatinoma in pancreas is seen in?
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.
Neurofibromatosis
Multiple endocrine neoplasia 2A
Turcot syndrome
Familial adenomatous polyposis
Pathophysiology - Pancreatic Endocrine Tumors