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2017 ; 13
(3
): 515-524
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English Wikipedia
Management of the hormonal syndrome of neuroendocrine tumors
#MMPMID28507564
Gut P
; Waligórska-Stachura J
; Czarnywojtek A
; Sawicka-Gutaj N
; B?czyk M
; Ziemnicka K
; Fischbach J
; Woli?ski K
; Kaznowski J
; Wrotkowska E
; Rucha?a M
Arch Med Sci
2017[Apr]; 13
(3
): 515-524
PMID28507564
show ga
Gastroenteropancreatic neuroendocrine tumors (GEP/NET) are unusual and rare
neoplasms that present many clinical challenges. They characteristically
synthesize store and secrete a variety of peptides and neuroamines which can lead
to the development of distinct clinical syndrome, however many are clinically
silent until late presentation with mass effects. Management strategies include
surgery cure and cytoreduction with the use of somatostatin analogues.
Somatostatin have a broad range of biological actions that include inhibition of
exocrine and endocrine secretions, gut motility, cell proliferation, cell
survival and angiogenesis. Five somatostatin receptors (SSTR1-SSTR5) have been
cloned and characterized. Somatostatin analogues include octreotide and
lanreotide are effective medical tools in the treatment and present selectivity
for SSTR2 and SSTR5. During treatment is seen disapperance of flushing,
normalization of bowel movements and reduction of serotonin and 5-hydroxyindole
acetic acid (5-HIAA) secretion. Telotristat represents a novel approach by
specifically inhibiting serotonin synthesis and as such, is a promising potential
new treatment for patients with carcinoid syndrome. To pancreatic functionig
neuroendocrine tumors belongs insulinoma, gastrinoma, glucagonoma and VIP-oma.
Medical management in patients with insulinoma include diazoxide which suppresses
insulin release. Also mTOR inhibitors may inhibit insulin secretion. Treatment of
gastrinoma include both proton pump inhibitors (PPIs) and histamine H2 - receptor
antagonists. In patients with glucagonomas hyperglycaemia can be controlled using
insulin and oral blood glucose lowering drugs. In malignant glucagonomas
smatostatin analogues are effective in controlling necrolytic migratory
erythemia. Severe cases of the VIP-oma syndrome require supplementation of fluid
losses. Octreotide reduce tumoral VIP secretion and control secretory diarrhoea.
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