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Multiple endocrine neoplasia: how many syndromes?
1Department of Medicine, Kansas University Medical School, Kansas City 66103.
American Journal of Medical Genetics
|November 1, 1990
Summary
Multiple Endocrine Neoplasia (MEN) syndromes are specific tumor associations. While three are recognized, other suggested syndromes lack strong evidence for reclassification as MEN.
Area of Science:
- Endocrinology
- Genetics
- Oncology
Background:
- Multiple Endocrine Neoplasia (MEN) refers to specific tumor associations constituting distinct syndromes.
- Three well-recognized MEN syndromes exist, all inherited as autosomal dominant traits and mapped to specific chromosomes.
Purpose of the Study:
- To critically examine evidence for purported new endocrine tumor syndromes or subtypes of existing MEN syndromes.
- To evaluate the validity of reclassifying other conditions as MEN syndromes.
Main Methods:
- Review and critical analysis of existing scientific literature and evidence.
- Examination of proposed associations between endocrine tumors and genetic syndromes.
Main Results:
- Only the association of pheochromocytomas and islet cell tumors appears reasonable, though some cases overlap with von Hippel-Lindau syndrome.
- No compelling evidence supports reclassifying conditions like von Hippel-Lindau syndrome, neurofibromatosis, or McCune-Albright syndrome as MEN syndromes.
Conclusions:
- The classification of MEN syndromes should adhere to established criteria.
- While awareness of endocrine abnormalities in other syndromes is important, reclassification as MEN is not warranted without strong evidence.
- Clinical vigilance for endocrine manifestations in related genetic disorders remains crucial.