Clinical Manifestations of Carcinoid Disease

Bax, N. D. S.; Woods, Helen Buckley; Batchelor, A.G.; Jennings, Marian

doi:10.1007/s002689900022

Cited by 43 publications

(29 citation statements)

References 29 publications

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“…The incidence of the carcinoid syndrome in these patients is highly dependent on the selected population. In nationwide surveys or cancer registries, 3-19% of patients with a small intestinal NET have the carcinoid syndrome, but incidence has been reported as high as 71% in specialized centers, treating more patients with advanced disease (Bax et al 1996, Janson et al 1997, Soga 2003, Niederle et al 2010, Halperin et al 2017.…”

Section: Serotonin and The Carcinoid Syndromementioning

confidence: 99%

Effect of hormone secretory syndromes on neuroendocrine tumor prognosis

Zandee

Kamp

Adrichem

et al. 2017

Endocrine-Related Cancer

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The treatment of hormone hypersecretory syndromes caused by neuroendocrine tumors (NETs) can be a major challenge. NETs originating from the small intestine often secrete serotonin causing flushing, diarrhea and valve fibrosis, leading to dehydration or heart failure in severe cases. NETs from the pancreas can secrete a wider variety of hormones, like insulin, glucagon and gastrin leading to distinct clinical syndromes. Historically mortality in patients with functioning NETs was high due to the complications caused by the hypersecretion of hormones. This has been reduced with several drugs: protonpump inhibitors decrease acid secretion caused by gastrinomas. Somatostatin analogs can inhibit the secretion of multiple hormones and these are now the cornerstone for treating patients with a gastroenteropancreatic NET. However, peptide receptor radionuclide therapy (PRRT) with radiolabeled somatostatin analogs and everolimus can also decrease symptoms of hypersecretion and increase progression-free survival. Several factors affect the survival in patients with a functioning NET. Complications of hypersecretion negatively impact survival; however, secretion of hormones is also often a sign of a well-differentiated NET and due to the symptoms, functioning NETs can be detected in an earlier stage suggesting a positive effect on prognosis. The effect on survival is also dependent on the type of hormone being secreted. This review aims to study the effect of hormone secretion on the prognosis of NETs with the contemporary treatments options available today.

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Section: Serotonin and The Carcinoid Syndromementioning

confidence: 99%

Effect of hormone secretory syndromes on neuroendocrine tumor prognosis

Zandee

Kamp

Adrichem

et al. 2017

Endocrine-Related Cancer

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“…The prevalence of flushing is reported to be about 80% [10, 11]. Flushing has been linked to several factors, e.g.…”

Section: Flushingmentioning

confidence: 99%

“…Diarrhea occurs in about 80% of patients [10, 11]. Several underlying mechanisms have been identified, although data on the relative contribution are lacking.…”

Section: Diarrheamentioning

confidence: 99%

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Complications of Midgut Carcinoid Tumors and Carcinoid Syndrome

Horst-Schrivers¹,

Wymenga²,

Links

et al. 2004

Neuroendocrinology

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The carcinoid syndrome, associated with carcinoid tumors of the midgut, consists of symptoms such as diarrhea, flushing, wheezing and cardiovascular symptoms. This review focuses on these symptoms and discusses therapeutic options. The symptoms are caused by the secretion of biogenic amines, polypeptides and other factors of which serotonin is the most prominent. However, diarrhea is also due to factors such as malabsorption. Besides antitumor therapy, more specific interventions such as serotonin receptor blockers can be useful. The carcinoid heart disease involves the tricuspid and pulmonary valve. In the pathogenesis, serotonin plays a central role. The therapeutic approach is mostly symptomatic. Other cardiovascular complications include bowel ischemia and hypertension. Pellagra and psychiatric symptoms are due to a depletion of tryptophan, which is consumed by the carcinoid tumor for serotonin synthesis. Finally, follow-up and clinical practice of patients with carcinoid tumors are discussed.

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“…It is administered by s.c. injection or i.v. infusion with s.c. dosing starting at 50-100 mg two to three times daily to a maximum daily dose of 3000 mg (Bax et al 1996). Other short-acting somatostatin analogues include lanreotide (Taylor et al 1988).…”

Section: Biotherapy Somatostatin Analoguesmentioning

confidence: 99%

Therapeutic management of patients with gastroenteropancreatic neuroendocrine tumours

Khan

Caplin²

2011

Endocrine-Related Cancer

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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.

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Clinical Manifestations of Carcinoid Disease

Cited by 43 publications

References 29 publications

Effect of hormone secretory syndromes on neuroendocrine tumor prognosis

Effect of hormone secretory syndromes on neuroendocrine tumor prognosis

Complications of Midgut Carcinoid Tumors and Carcinoid Syndrome

Therapeutic management of patients with gastroenteropancreatic neuroendocrine tumours

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