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Multiple endocrine neoplasia type 1- presenting multiple lipomas and hypoglycemia onset
Jianzhong Li1, Lixian Zeng, Yidong Yang
1Department of Gastroenterology, Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
The American Journal of Case Reports
|April 10, 2013
Summary
Multiple endocrine neoplasia type 1 (MEN1), or Wermer syndrome, is a genetic disorder causing tumors. This case highlights how lipomas, insulinoma, and gastrinoma can indicate MEN1 diagnosis.
Area of Science:
- Endocrinology
- Oncology
- Genetics
Background:
- Multiple endocrine neoplasia type 1 (MEN1), also known as Wermer syndrome, is an autosomal dominant disorder.
- MEN1 is characterized by tumors in the parathyroid glands, anterior pituitary, and endocrine pancreas.
Purpose of the Study:
- To report a case of MEN1 with unusual manifestations.
- To highlight key clinical features that may suggest a diagnosis of MEN1.
Main Methods:
- Case report of a 44-year-old woman with MEN1.
- Clinical presentation included insulinoma, pituitary tumors, parathyroidoma, multiple lipomas, and gastrinoma with duodenal ulcers.
- Immunohistochemical analysis of pancreatic tumors for insulin and glucagon.
Main Results:
- The patient presented with recurrent upper gastrointestinal bleeding and hypoglycemia.
- Diagnosis of insulinoma and gastrinoma was confirmed.
- Pancreatic tumor cells were predominantly insulin-positive and glucagon-negative.
Conclusions:
- Multiple lipomas, insulinoma, and gastrinoma can be important diagnostic clues for MEN1.
- Early recognition of these signs can facilitate timely diagnosis and management of MEN1.
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