Multiple endocrine and other organ neoplasia syndromes

Paul J. Newey; Rajesh V. Thakker

doi:10.1016/B978-0-443-13683-2.00018-9

Multiple endocrine and other organ neoplasia syndromes

Paul J. Newey
, Rajesh V. Thakker

Diabetes Endocrinology and Reproductive Biology

Research output: Chapter in Book/Report/Conference proceeding › Chapter

Abstract

The multiple endocrine and other organ neoplasia syndromes (MEONSs) are a heterogeneous group of monogenic disorders characterized by the development of endocrine neoplasia alongside a range of nonendocrine tumors. The MEONS include the hyperparathyroidism-jaw tumor syndrome, Von Hippel-Lindau disease, Carney Complex, Neurofibromatosis type 1, Cowden syndrome, and McCune-Albright syndrome. Each of these is typically inherited as an autosomal dominant disorder, except MAS, which is due to a mosaic expression of a postzygotic somatic GNAS variant. The MEONS are associated with a range of skeletal diseases that include osteoporosis, scoliosis, pseudoarthroses, long bone and spinal dysplasias, ossifying tumors, and osteochondromyxomas.

Original language	English
Title of host publication	Genetics of Bone Biology and Skeletal Disease
Editors	Rajesh V Thakker, Michael P Whyte, John A Eisman
Place of Publication	London
Publisher	Elsevier
Chapter	43
Pages	947-962
Number of pages	16
Edition	3
ISBN (Electronic)	9780443136832
ISBN (Print)	9780443136849
DOIs	https://doi.org/10.1016/B978-0-443-13683-2.00018-9
Publication status	Published - 25 Mar 2026

Keywords

endocrine neoplasia
Monogenic
pathogenic variant

ASJC Scopus subject areas

General Agricultural and Biological Sciences
General Biochemistry,Genetics and Molecular Biology

Access to Document

10.1016/B978-0-443-13683-2.00018-9

Cite this

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abstract = "The multiple endocrine and other organ neoplasia syndromes (MEONSs) are a heterogeneous group of monogenic disorders characterized by the development of endocrine neoplasia alongside a range of nonendocrine tumors. The MEONS include the hyperparathyroidism-jaw tumor syndrome, Von Hippel-Lindau disease, Carney Complex, Neurofibromatosis type 1, Cowden syndrome, and McCune-Albright syndrome. Each of these is typically inherited as an autosomal dominant disorder, except MAS, which is due to a mosaic expression of a postzygotic somatic GNAS variant. The MEONS are associated with a range of skeletal diseases that include osteoporosis, scoliosis, pseudoarthroses, long bone and spinal dysplasias, ossifying tumors, and osteochondromyxomas.",

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KW - pathogenic variant

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Multiple endocrine and other organ neoplasia syndromes

Abstract

Keywords

ASJC Scopus subject areas

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