Neuroendocrine tumours.

2004 ◽  
Vol 11 (1) ◽  
pp. 1-18 ◽  
Author(s):  
M T Barakat ◽  
K Meeran ◽  
S R Bloom
Keyword(s):  
Neuroendocrine Tumours ◽  
Secretory Granules ◽  
Symptom Control ◽  
Medullary Carcinoma ◽  
Somatostatin Analogues ◽  
Pharmacological Therapy ◽  
Neuroendocrine Cells ◽  
Hormone Production ◽  
Neuropeptide Hormone ◽  
Gastroenteropancreatic Neuroendocrine Tumours

Neuroendocrine tumours are a heterogeneous group including, for example, carcinoid, gastroenteropancreatic neuroendocrine tumours, pituitary tumours, medullary carcinoma of the thyroid and phaeochromocytomas. They have attracted much attention in recent years, both because they are relatively easy to palliate and because they have indicated the chronic effect of the particular hormone elevated. As neuroendocrine phenotypes became better understood, the definition of neuroendocrine cells changed and is now accepted as referring to cells with neurotransmitter, neuromodulator or neuropeptide hormone production, dense-core secretory granules, and the absence of axons and synapses. Neuroendocrine markers, particularly chromogranin A, are invaluable diagnostically. Study of several neuroendocrine tumours has revealed a genetic etiology, and techniques such as genetic screening have allowed risk stratification and prevention of morbidity in patients carrying the particular mutation. Pharmacological therapy for these often slow-growing tumours, e.g. with somatostatin analogues, has dramatically improved symptom control, and radiolabelled somatostatin analogues offer targeted therapy for metastatic or inoperable disease. In this review, the diagnosis and management of patients with carcinoid, gut neuroendocrine tumours, multiple endocrine neoplasia types 1 and 2, and isolated phaeochromocytoma are evaluated.

scholarly journals Gastroenteropancreatic neuroendocrine tumours: the current incidence and staging based on the WHO and European Neuroendocrine Tumour Society classification: an analysis based on prospectively collected parameters

2010 ◽  
Vol 17 (4) ◽  
pp. 909-918 ◽  
Author(s):  
Martin B Niederle ◽  
Monika Hackl ◽  
Klaus Kaserer ◽  
Bruno Niederle
Keyword(s):  
Digestive Tract ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Tnm Staging ◽  
Incidence Rates ◽  
Neuroendocrine Cells ◽  
Malignant Tumours ◽  
Main Site ◽  
Well Differentiated ◽  
Gastroenteropancreatic Neuroendocrine Tumours

As incidence data on gastroenteropancreatic neuroendocrine tumours (GEP-NETs) have so far only been retrospectively obtained and based on inhomogeneous material, we conducted a prospective study in Austria collecting all newly diagnosed GEP-NETs during 1 year. Using the current WHO classification, the tumor, nodes, metastases (TNM) staging and Ki67 grading and the standard diagnostic procedure proposed by the European Neuroendocrine Tumor Society (ENETS), GEP-NETs from 285 patients (male: 148; female: 137) were recorded. The annual incidence rates were 2.51 per 100 000 inhabitants for men, 2.36 per 100 000 for women. The stomach (23%) was the main site, followed by appendix (21%), small intestine (15%) and rectum (14%). Patients with appendiceal tumours were significantly younger than patients with tumours in any other site. About 46.0% were classified as benign, 15.4% as uncertain, 31.9% as well differentiated malignant and 6.7% as poorly differentiated malignant. Patients with benign or uncertain tumours were significantly younger than patients with malignant tumours. Among the malignant tumours of the digestive tract, 1.49% arose from neuroendocrine cells. For malignant gastrointestinal NETs, the incidence was 0.80 per 100 000: 40.9% were ENETS stage I, 23.8% stage II, 11.6% stage III and 23.8% stage IV. The majority (59.7%) were grade 1, 31.2% grade 2 and 9.1% grade 3. NETs of the digestive tract are more common than previously reported; the majority show benign behaviour, are located in the stomach and are well differentiated. G3 tumours are very rare.

scholarly journals Somatostatin analogues in acromegaly and gastroenteropancreatic neuroendocrine tumours: past, present and future

2016 ◽  
Vol 23 (12) ◽  
pp. R551-R566 ◽  
Author(s):  
Kjell Öberg ◽  
Steven W J Lamberts
Keyword(s):  
Medical Therapy ◽  
Neuroendocrine Tumours ◽  
Clinical Success ◽  
Serum Insulin ◽  
Therapeutic Option ◽  
Hormone Secretion ◽  
Somatostatin Analogues ◽  
Somatostatin Analogue ◽  
Prolonged Release ◽  
Gastroenteropancreatic Neuroendocrine Tumours

Acromegaly is a hormonal disorder that arises when the pituitary gland secretes excess growth hormone (GH), which in turn stimulates a concomitant increase in serum insulin-like growth factor 1 (IGF-1) levels. Gastroenteropancreatic neuroendocrine tumours (GEP-NET) constitute a heterogeneous group of tumours that can secrete serotonin and a variety of peptide hormones that may cause characteristic symptoms known as carcinoid syndrome or other symptoms and hormonal hypersecretion syndromes depending on the tumour’s site of origin. Current medical therapy for the treatment of acromegaly and GEP-NET involves the administration of somatostatin analogues that effectively suppress excess hormone secretion. After its discovery in 1979, octreotide became the first synthetic biologically stable somatostatin analogue with a short-acting formulation of octreotide introduced into clinical practice in the late 1980s. Lanreotide, another somatostatin analogue, became available in the mid-1990s initially as a prolonged-release formulation administered every 10 or 14 days. Long-acting release formulations of both octreotide (Sandostatin LAR and Novartis) and lanreotide (Somatuline Autogel, Ipsen), based on microparticle and nanoparticle drug-delivery technologies, respectively, were later developed, which allowed for once-monthly administration and improved convenience. First-generation somatostatin analogues remain one of the cornerstones of medical therapy in the management of pituitary and GEP-NET hormone hypersecretion, with octreotide having the longest established efficacy and safety profile of the somatostatin analogue class. More recently, pasireotide (Signifor), a next-generation multireceptor-targeted somatostatin analogue, has emerged as an alternative therapeutic option for the treatment of acromegaly. This review summarizes the development and clinical success of somatostatin analogues.

Therapeutic management of patients with gastroenteropancreatic neuroendocrine tumours

2011 ◽  
Vol 18 (S1) ◽  
pp. S53-S74 ◽  
Author(s):  
Mohid S Khan ◽  
Martyn E Caplin
Keyword(s):  
Neuroendocrine Tumours ◽  
Mammalian Target Of Rapamycin ◽  
Hepatic Metastases ◽  
Somatostatin Analogues ◽  
Medical Treatments ◽  
Emerging Therapies ◽  
Specialist Centre ◽  
Specialist Nurses ◽  
Gastroenteropancreatic Neuroendocrine Tumours ◽  
Consensus Expert

Patients with neuroendocrine tumours (NETs) are best managed in a specialist centre as part of a multidisciplinary team comprising gastroenterologists, oncologists, endocrinologists, gastrointestinal and hepatopancreaticobiliary surgeons, pathologists, nuclear medicine physicians and technicians, radiologists, specialist nurses, pharmacists, biochemists and dieticians. This should ideally be led by a clinician with experience and interest in NETs. Although the number of medical treatments and clinical trials has increased in the decade, there is still a lack of prospective randomised trials; thus, management is mainly based on limited often single-centre studies, although there are now formal guidelines based on consensus expert opinion. We have outlined the current optimal management of patients with NETs. We have reviewed therapeutic options including surgery, somatostatin analogues and other biotherapies and peptide receptor-targeted therapy. We have discussed the challenge in managing hepatic metastases including hepatic artery embolisation, ablation and orthotopic liver transplant. In addition, we have briefly reviewed the emerging therapies such as the mammalian target of rapamycin and angiogenic inhibitors and the newer somatostatin analogues.

scholarly journals Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours

2016 ◽  
Vol 23 (9) ◽  
pp. R423-R436 ◽  
Author(s):  
Georgios K Dimitriadis ◽  
Martin O Weickert ◽  
Harpal S Randeva ◽  
Gregory Kaltsas ◽  
Ashley Grossman
Keyword(s):  
Medical Management ◽  
Tumour Growth ◽  
Neuroendocrine Tumours ◽  
Somatostatin Analogues ◽  
Binding Activity ◽  
Biologically Active ◽  
Morbidity And Mortality ◽  
Clinical Syndromes ◽  
Long Acting ◽  
Gastroenteropancreatic Neuroendocrine Tumours

Although recent epidemiological evidence indicates that the prevalence of non-functioning gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) is rising, a significant number of GEP-NETs still present with symptoms related to the secretion of biologically active substances leading to the development of distinct clinical syndromes. In the past, these syndromes were associated with substantial morbidity and mortality due to the lack of specific therapies; however, since the introduction of long-acting somatostatin analogues and medications such as proton pump inhibitors, their control has been greatly improved. As a result, nowadays, the main cause of morbidity and mortality in GEP-NETs is mostly directly related to tumour growth and the extent of metastatic disease. However, in some patients with functioning tumours and extensive disease, control of the secretory syndrome still remains problematic, necessitating the employment of several cytoreductive techniques, which may not always be sufficient. Recently, new agents directed against tumour growth, or exerting increased binding activity to receptors expressed in these tumours, or interfering with the synthetic pathway of some of the compounds secreted by these tumours, have been developed. Since there are no specific guidelines addressing the totality of the management of the secretory syndromes related to GEP-NETs, this review aims at critically analysing the medical management of previously recognised secretory syndromes; it also addresses areas of uncertainty, assesses the newer therapeutic developments and also addresses recently described but poorly characterised secretory syndromes related to GEP-NETs.

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