Multiple Endocrine Neoplasia in Childhood: An Update on Diagnosis, Screening, Management and Treatment

Multiple endocrine neoplasia (MEN) is a group of heterogenous syndromes characterized by the occurrence of two or more endocrine gland tumors in a patient or related individuals in the same family. They are inherited in an autosomal dominant fashion and are highly penetrant. There are three types of...

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Bibliographic Details
Published in:Endocrines 2022-02, Vol.3 (1), p.76-91
Main Authors: Jacob, Marianne, Rowland, Dustin, Lekarev, Oksana, Ergun-Longmire, Berrin
Format: Article
Language:English
Subjects:
Acids
Age
Asymptomatic
Childhood
Cyclin-dependent kinases
familial medullary thyroid carcinoma
Genotype & phenotype
Hypercalcemia
Hyperplasia
Hypoglycemia
Insulin
Males
MEN1
MEN2A
MEN2B
MEN4
multiple endocrine neoplasia
Mutation
Neuroendocrine tumors
Pancreas
Thyroid cancer
Thyroid gland
Ulcers
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Description
Summary:Multiple endocrine neoplasia (MEN) is a group of heterogenous syndromes characterized by the occurrence of two or more endocrine gland tumors in a patient or related individuals in the same family. They are inherited in an autosomal dominant fashion and are highly penetrant. There are three types of MEN syndromes: MEN type 1 (MEN1), MEN type 2 (MEN2), and MEN type 4 (MEN4). MEN2 is further divided into MEN2A, MEN2B (formerly known MEN3), and familial medullary thyroid carcinoma (FMTC). Although MEN syndromes are rare, it is crucial to identify individuals at risk for potentially life-threatening neoplasias. This review article provides an update on each MEN syndrome, its genetics, diagnosis, and management in children.
ISSN:2673-396X
2673-396X
DOI:10.3390/endocrines3010007