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Familial syndromes associated with neuroendocrine tumours
#MMPMID26557756
Gut P
; Komarowska H
; Czarnywojtek A
; Waligórska-Stachura J
; B?czyk M
; Ziemnicka K
; Fischbach J
; Wrotkowska E
; Rucha?a M
Contemp Oncol (Pozn)
2015[]; 19
(3
): 176-83
PMID26557756
show ga
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.
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