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Multiple endocrine neoplasia.

D R Caruso¹, T M O'Dorisio, E L Mazzaferri

¹Ohio State University School of Medicine, Columbus.

Current Opinion in Oncology

|February 1, 1991

Summary

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Multiple endocrine neoplasia (MEN) syndromes involve inherited endocrine tumors. Genetic markers on chromosomes 11 (MEN-1) and 10 (MEN-2) aid in identifying at-risk individuals for these rare genetic disorders.

Area of Science:

Endocrinology
Genetics
Oncology

Background:

Multiple endocrine neoplasia (MEN) syndromes are inherited disorders characterized by the development of tumors in endocrine glands.
MEN syndromes have distinct subtypes, MEN-1 and MEN-2, each associated with specific tumor profiles and genetic loci.

Purpose of the Study:

To review the clinical presentation, diagnostic approaches, and management strategies for MEN-1 and MEN-2 syndromes.
To highlight advancements in genetic analysis for identifying at-risk family members.

Main Methods:

Review of clinical data on tumor frequency and symptomatology in MEN-1.
Discussion of surgical and medical treatment efficacy, including octreotide for MEN-1.
Description of biochemical screening and imaging techniques for MEN-2 neoplasms.

Related Experiment Videos

Analysis of genetic linkage studies for MEN-1 and MEN-2 genes.

Main Results:

MEN-1 involves parathyroid, pituitary, and pancreatic tumors, with genetic linkage to chromosome 11.
MEN-2 is characterized by medullary thyroid carcinoma and pheochromocytoma, with genetic linkage to chromosome 10.
Effective management strategies include surgery, symptomatic therapy, and genetic screening.

Conclusions:

Early identification and intervention are crucial for managing MEN syndromes.
Genetic markers significantly improve the ability to identify at-risk individuals for MEN-1 and MEN-2.
Advancements in screening and genetic analysis enhance patient outcomes for these familial endocrine tumor syndromes.