Management of the hormonal syndrome of neuroendocrine tumors

Abstract

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.

Carcinoid Syndrome: A Review

“…It lasts only a few minutes and can appear many times in a day. It may be triggered by alcohol, exercise, or tyramine-containing foods (chocolate, walnuts, bananas) [19]. It is not associated with sweating.…”

“…Cytotoxic treatment is the first-line treatment for malignant neuroendocrine tumors in the pancreas and for gastric carcinoids if the Ki67 antibody level is greater than 10%. It may be a second-line treatment if other means of treatment fail [19].…”

The management of neuroendocrine tumours: A nutritional viewpoint

“…telotristat ethyl) are under investigation (Kulke et al 2017). In patients refractory to SSAs, as well as in patients who experience the GI side effects of SSAs, anti-motility agents (loperamide and opiates) may be used for symptomatic improvement of NETs-related diarrhoea (Gut et al 2017). They should represent the initial therapy in patients with medullary thyroid cancer and diarrhoea (Schlumberger et al 2012).…”

Ovarian Sertoli-Leydig cell tumour with α-fetoprotein-producing intestinal glandular cells. Clinical case and short review of basic literature

“…We presented a case of Sertoli-Leydig cell tumour with heterogeneous elements of glandular colonic cells producing α-fetoprotein. So far the medical literature lists approximately 30 similar cases [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25]. Sertoli-Leydig cell tumours most commonly (75%) occur in young females up to 30 years of age [26,27].…”