Multiple Endocrine Neoplasia
MEN 1 / Wermer syndrome:
Menin gene: chromosome 11
3 P: Par>Pan>Pit
Pituitary adenoma – Most Common: Prolactinoma
Parathyroid adenoma
Pancreatic endocrine tumor
• Non-functioning pancreatic tumors (most common overall)
• Functioning pancreatic tumors :
• Gastrinoma -50% (most common among
• Insulinoma - 30%
• Glucagonoma
• ViPoma (least common)
Around 90% of gastrinomas are found in this triangle.
The most frequently observed cutaneous manifestation of MEN1 syndrome is angiofibroma.
Its boundaries are as follows:
• Cystic duct
• Junction of the 2nd and 3dr parts of the duodenum
• Junction of the body and neck of the pancreas
The most frequently observed cutaneous manifestation of MEN1 syndrome is angiofibroma.
Cutaneous tumors in MEN1 Syndrome are:
• Angiofibroma - 85%
• Collagenoma - 70%
• Lipoma – 33%
Insulinoma is the second most common functioning enteropancreatic tumor in MEN1 syndrome.
Investigation of choice is 72-hour fasting glucose and serum insulin levels. These parameters are
measured when the patient develops hypoglycemic symptoms during a supervised 72-hour fasting
period.
The treatment of choice is surgery, ranging from enucleation of a single tumor to pancreatectomy.
Non surgical management of insulinoma includes frequent consumption of carbohydrate-rich meals,
octreotide or diazoxide, which are not always successful.
Selpercatinib, a kinase inhibitor was recently approved by the FDA. It is used to treat tumors with a
mutation in the RET gene. These are non- small cell lung cancer, medullary thyroid cancer, and other
types of thyroid cancers in patients with a mutation in the RET gene.
