Familial syndromes associated with neuroendocrine tumours

Paweł Gut; Hanna Komarowska; Agata Czarnywojtek; Joanna Waligórska-Stachura; Maciej Bączyk; Katarzyna Ziemnicka; Jakub Fischbach; Elżbieta Wrotkowska; Marek Ruchała

doi:10.5114/wo.2015.52710

eISSN: 1897-4309
ISSN: 1428-2526

Contemporary Oncology/Współczesna Onkologia

Current issue Archive Manuscripts accepted About the journal Supplements Addendum Special Issues Editorial board Reviewers Abstracting and indexing Subscription Contact Instructions for authors Publication charge Ethical standards and procedures

Editorial System

Submit your Manuscript

Editorial Policies

Sarajevo Declaration on Integrity and Visibility of Scholarly Publications

3/2015
vol. 19

Send email

Copy url:

abstract:

Review paper

Familial syndromes associated with neuroendocrine tumours

Paweł Gut

,

Hanna Komarowska

,

Agata Czarnywojtek

,

Joanna Waligórska-Stachura

,

Maciej Bączyk

,

Katarzyna Ziemnicka

,

Jakub Fischbach

,

Elżbieta Wrotkowska

,

Marek Ruchała

Contemp Oncol (Pozn) 2015; 19 (3): 176–183

DOI: https://doi.org/10.5114/wo.2015.52710

Online publish date: 2015/07/08

View full text Get citation

PlumX metrics:

Neuroendocrine tumours may be associated with familial syndromes. At least eight inherited syndromes predisposing to endocrine neoplasia have been identified. Two of these are considered to be major factors predisposing to benign and malignant endocrine tumours, designated multiple endocrine neoplasia type 1 and type 2 (MEN1 and MEN2). Five other autosomal dominant diseases show more heterogeneous clinical patterns, such as the Carney complex, hyperparathyroidism-jaw tumour syndrome, Von Hippel-Lindau syndrome (VHL), neurofibromatosis type 1 (NF1) and tuberous sclerosis. The molecular and cellular interactions underlying the development of most endocrine cells and related organs represent one of the more complex pathways not yet to be deciphered. Almost all endocrine cells are derived from the endoderm and neuroectoderm. It is suggested that within the first few weeks of human development there are complex interactions between, firstly, the major genes involved in the initiation of progenitor-cell differentiation, secondly, factors secreted by the surrounding mesenchyme, and thirdly, a series of genes controlling cell differentiation, proliferation and migration. Together these represent a formula for the harmonious development of endocrine glands and tissue.

keywords:

Familial syndromes associated with neuroendocrine tumours

Paweł Gut , Hanna Komarowska , Agata Czarnywojtek , Joanna Waligórska-Stachura , Maciej Bączyk , Katarzyna Ziemnicka , Jakub Fischbach , Elżbieta Wrotkowska , Marek Ruchała

neuroendocrine tumours, familial syndromes

Paweł Gut

,

Hanna Komarowska

,

Agata Czarnywojtek

,

Joanna Waligórska-Stachura

,

Maciej Bączyk

,

Katarzyna Ziemnicka

,

Jakub Fischbach

,

Elżbieta Wrotkowska

,

Marek Ruchała