SYNDROMES OF MULTIPLE ENDOCRINE NEOPLASIAS
Journal Title: Праці Наукового товариства ім. Шевченка - Year 2015, Vol 43, Issue 2
Abstract
The term syndrome o f multiple endocrine neoplasia (MEN) unites a group o f diseases that include tumors o f neuroendocrine origin and/or hyperplasia o f two or more endocrine organs. MEN syndromes have a number o f common signs, in particular: different metabolic and endocrine dysfunctions; symptoms caused by hypersecretion o f one or several hormones; they are often accompanied by dysplasia o f other organs; presence o f several foci o f hyperplasia or neoplastic growth; they are caused by genetic defects and autosomal dominant inheritance; high penetrance; dependence o f clinical and laboratory indicators on the stage o f tumor development. In fifty percent o f cases, MEN apears sporadically, i.e. it is caused by a mutation in germ or somatic cells. The risk o f disease incidence in patient’s children is 50%. Genealogical, cytogenetic, and molecular genetic research found mutations that underlie the known types o f MEN syndrome: in case o f MEN-1 it is the mutation of tumor growth suppressor gene (11q13) that codes menin protein which ensures regulation ofproliferation o f neural crest derivative cells; in case o f MEN-2 it is the mutation of RET proto-oncogene which is localized on the long arm o f the 10th chromosome. The majority of tumors that belong to MEN are neuroendocrine and stem from APUD-system cells. Any type o f APUD-system cells (islet cells o f the pancreas, neuroendocrine cells o f the respiratory system and gastrointestinal tract, and parafollicular cells o f the thyroid gland) can be the source of development o f tumors called apudomas. The choice o f treatment method in case of MEN syndromes depends on clinical signs, extensiveness o f neoplastic process, and possible complications. Timely diagnosis and definitive treatment improve the patient’s prognosis.
Authors and Affiliations
Ruslan LITVINYAK, Borys BILYNSKYY
Keywords
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