Neuroendocrine disorders

2021 ◽  
pp. 627-650
Author(s):  
Karin Bradley

This chapter begins by covering the neuroendocrine system, then neuroendocrine tumours. Carcinoid syndrome, insulinomas, gastrinomas, glucagonomas, and VIPomas are all described, as well as other rare functional pancreatic NETs.

Author(s):  
Rajaventhan Srirajaskanthan ◽  
Martyn E. Caplin ◽  
Humphrey Hodgson

Neuroendocrine tumours (NETs) are derived from cells of the diffuse neuroendocrine system, which are present in organs throughout the body. Originally, Pearse proposed that tumours develop from migration of cells from the neural crest; however, it is now thought that the tumour cells are derived from multipotent stem cells (1). The term ‘karzinoide’ (meaning carcinoma like) was initially introduced by Siegfried Oberndorfer in 1907 (2). The term carcinoid tumour has historically been used; however, with advances in the understanding of the tumour biology, and the recent WHO classification, the term NET or endocrine tumour is considered more appropriate, and more details are given in the historical introduction in Chapter 6.1.


2014 ◽  
Vol 170 (2) ◽  
pp. K5-K9 ◽  
Author(s):  
Louise A Lynggård ◽  
Eigil Husted Nielsen ◽  
Peter Laurberg

BackgroundNeuroendocrine tumours are most frequently located in the gastrointestinal organ system or in the lungs, but they may occasionally be found in other organs.CaseWe describe a 56-year-old woman suffering from a carcinoid syndrome caused by a large serotonin-secreting pituitary tumour. She had suffered for years from episodes of palpitations, dyspnoea and flushing. Cardiac disease had been suspected, which delayed the diagnosis, until blood tests revealed elevated serotonin and chromogranin A in plasma. The somatostatin receptor (SSR) scintigraphy showed a single-positive focus in the region of the pituitary gland and MRI showed a corresponding intra- and suprasellar heterogeneous mass. After pre-treatment with octreotide leading to symptomatic improvement, the patient underwent trans-cranial surgery with removal of the tumour. This led to a clinical improvement and to a normalisation of SSR scintigraphy, as well as serotonin and chromogranin A levels.ConclusionTo our knowledge, this is the first reported case of a serotonin-secreting tumour with a primary location in the pituitary.


2014 ◽  
Vol 155 (5) ◽  
pp. 194-198
Author(s):  
Andrea Uhlyarik ◽  
Erika Lahm ◽  
József Vachaja ◽  
Zsuzsanna Pápai

Although the incidence of neuroendocrine tumours is low, their prevalence is high due to the usually slow course of the disease. Between July 1, 2008 and July 1, 2013 the authors evaluated 56 patients with well-differentiated or moderately differentiated neuroendocrine tumours; 36 patients with metastatic disease underwent treatment while 17 patients who had tumour resection were followed without additional treatment. All patients with metastatic disease received long acting octreotide, and additional therapy was based on the site of origin, grade of differentiation, Ki67 index, and focal labelling of the tumours during somatostatin-receptor or metaiodo-benzyl-guanidine scintigraphy. The authors present a detailed case history of a patient with carcinoid syndrome due to a metastatic midgut neuroendocrine tumour, who received long acting octreotide and peptide receptor radionuclide treatment. In this patient an objective tumour response was reached in addition to the resolution of symptoms of carcinoid syndrome. The authors conclude that the case history confirms previous observations showing that long acting octreotide combined with peptide receptor radionuclide treatment may provide long survival with good quality of life in a patient with metastatic midgut neuroendocrine tumour accompanied with carcinoid syndrome. Orv. Hetil., 2014, 155(5), 194–198.


Author(s):  
A. Custodio ◽  
P. Jimenez-Fonseca ◽  
A. Carmona-Bayonas ◽  
M. J. Gomez ◽  
M. I. Del Olmo-García ◽  
...  

Abstract Purpose To report healthcare resource use and associated costs in controlled versus uncontrolled carcinoid syndrome (CS) in patients with neuroendocrine tumours. Methods A cross-sectional, non-interventional multicentre study was conducted with retrospective data analysis. Resource use was compared between two patient groups: those with controlled CS (> 12 months with no uncontrolled CS episodes) and uncontrolled CS (< 12 months since last uncontrolled episode). Patients were matched for age, sex, and origin and grade of tumour. When no matching patients were available, data from deceased patients were used. Information on healthcare resource use came from review of medical records, patient history and physician reports. Working capacity was assessed using the Work Productivity and Activity Impairment General Health questionnaire. Results Twenty-six university hospitals in Spain participated, between July 2017 and April 2018. 137 patients were enrolled; 104 were analysed (2 groups of 52). Patients with uncontrolled CS had 10 times more emergency department (ED) visits (mean 1.0 vs 0.10 visits; P = 0.0167), were more likely to have a hospital admission (40.4% vs 19.2%; P = 0.0116) and had longer hospital stays (mean 7.87 vs 2.10 days; P = 0.0178) than those with controlled CS. This corresponded to higher annual hospitalisation costs (mean €5511.59 vs €1457.22; P = 0.028) and ED costs (€161.25 vs €14.85; P = 0.0236). The mean annual total healthcare costs were 60.0% higher in patients with uncontrolled than controlled CS (P = NS). Conclusion This study quantifies higher health resource use, and higher hospitalisation and ED costs in patients with uncontrolled CS. Better control of CS may result 3in lower medical costs.


Medicine ◽  
2013 ◽  
Vol 41 (10) ◽  
pp. 566-569 ◽  
Author(s):  
Channa N. Jayasena ◽  
Waljit S. Dhillo

Author(s):  
Michael P Rogers ◽  
Sarah Thomas ◽  
Leelakrishna Nallamshetty ◽  
Robert Hooker

Abstract Neuroendocrine tumours are rare neoplasms typically arising in the gastrointestinal tract that may result in carcinoid syndrome and/or acquired valvular dysfunction. Herein, we present a unique case of a 68-year-old asymptomatic woman with a primary left ventricular neuroendocrine tumour.


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