Associated with Menin tumor suppressor gene on chromosome 11
Parathyroid adenoma à hypercalcemia
Pituitary adenoma à prolactinoma, Cushing's, acromegaly
Pancreatic islet cell tumors à Insulinoma, Gastrinoma, VIPoma, Glucagonoma
May also be associated with cutaneous tumors and carcinoid
Screening for MEN I
Hyperparathyroidism is the earliest manifestation, and serum Ca is the best way to screen families
Other methods: prolactin, GH/IGF-1, gastrin & pancreatic enzymes
Imaging: periodic imaging of pancreas, adrenal and pituitary
Multiple Endocrine Neoplasia (MEN) II
Associated with RET protooncogene mutation on chromosome 10
2 types: MEN IIA & MEN IIB
Both MEN IIA & MEN IIB have Pheochromocytoma and Medullary thyroid cancer
MEN IIA: Parathyroid hyperplasia (à high calcium)
MEN IIB: Mucosal neuroma lips, tongue, GI) and Marfanoid (slim body habitus)
Screening for MEN II
Screen asymptomatic family members with ret genetic testing
Hyperparathyroidism: serum ca
Pheochromocytoma: metanephrine or catecholamine
Medullary thyroid carcinoma: total thyroidectomy is recommended in early childhood once the genetic mutation identified, Calcitonin work as tumor marker
