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2015 ; 10
(3
): 127-34
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Clinical features of gastroenteropancreatic tumours
#MMPMID26516377
Gut P
; Czarnywojtek A
; B?czyk M
; Ziemnicka K
; Fischbach J
; Wrotkowska E
; Rucha?a M
Prz Gastroenterol
2015[]; 10
(3
): 127-34
PMID26516377
show ga
Gastroenteropancreatic (GEP) endocrine tumours (carcinoids and pancreatic islet
cell tumours) are composed of multipotent neuroendocrine cells that exhibit a
unique ability to produce, store, and secrete biologically active substances and
cause distinct clinical syndromes. The classification of GEP tumours as
functioning or non-functioning is based on the presence of symptoms that
accompany these syndromes secondary to the secretion of hormones, neuropeptides
and/or neurotransmitters (functioning tumours). Non-functioning tumours are
considered to be neoplasms of neuroendocrine differentiation that are not
associated with obvious symptoms attributed to the hypersecretion of
metabolically active substances. However, a number of these tumours are either
capable of producing low levels of such substances, which can be detected by
immunohistochemistry but are insufficient to cause symptoms related to a clinical
syndrome, or alternatively, they may secrete substances that are either
metabolically inactive or inappropriately processed. In some cases, GEP tumours
are not associated with the production of any hormone or neurotransmitter. Both
functioning and non-functioning tumours can also produce symptoms due to mass
effects compressing vital surrounding structures. Gastroenteropancreatic tumours
are usually classified further according to the anatomic site of origin: foregut
(including respiratory tract, thymus, stomach, duodenum, and pancreas), midgut
(including small intestine, appendix, and right colon), and hindgut (including
transverse colon, sigmoid, and rectum). Within these subgroups the biological and
clinical characteristics of the tumours vary considerably, but this
classification is still in use because a significant number of previous studies,
mainly observational, have used it extensively.