Treatment of the Malignant Carcinoid Syndrome

Kvols, Larry K.; Moertel, Charles G.; O’Connell, Michael J.; Schutt, Allan J.; Rubin, Joseph; Hahn, Richard G.

doi:10.1056/nejm198609113151102

Cited by 758 publications

(100 citation statements)

References 10 publications

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“…Subsequent studies using s.c. octreotide at doses ranging from 50 to 1000 μg, but occasionally up to 3 mg, 2-3 times per day, showed that significant symptomatic improvement of CS was obtained. An analysis of 10 studies that evaluated more than 300 patients with CS treated with s.c. octreotide, with doses ranging between 150 and 3000 μg (median dose 450 μg), showed 70% control of both flushing and diarrhoea (Kvols et al 1986, Vinik & Moattari 1989, Anthony et al 1993, Janson & Oberg 1993, Saltz et al 1993, Arnold et al 1996, di Bartolomeo et al 1996, Rubin et al 1999, O'Toole et al 2000, Aparicio et al 2001. A randomised study comparing daily s.c. injections of short-acting octreotide to octreotide LAR at various doses every 4 weeks demonstrated an at least equivalent symptomatic control (Rubin et al 1999).…”

Section: :9mentioning

confidence: 99%

Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours

Dimitriadis¹,

Weickert²,

Randeva³

et al. 2016

Endocrine-Related Cancer

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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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Section: :9mentioning

confidence: 99%

Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours

Dimitriadis¹,

Weickert²,

Randeva³

et al. 2016

Endocrine-Related Cancer

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“…Long-acting somatostatin analogues as octreotide acetate and lanreotide have been repeatedly shown to improve or even normalize diarrhoea and flushing in patients with carcinoid syndrome [10, 12, 13, 14, 15]during both short- and long-term treatment. Symptom relief is correlated with reduction of hormonal markers.…”

Section: Control Of Symptoms In Patients With Functionally Active Neumentioning

confidence: 99%

Treatment of Neuroendocrine GEP Tumours with Somatostatin Analogues

Arnold¹,

Simon²,

Wied³

2000

Digestion

146

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Background: Somatostatin and its long-acting analogues are effective in symptom control in patients with functionally active neuroendocrine GEP tumours. Several in vitro and in vivo reports suggest that they are also able to control tumour growth. Methods: Critical review of published data on the effect of long-acting somatostatin analogues on symptom and growth control in patients with metastatic neuroendocrine GEP tumours. Results: With the exception of insulinoma and gastrinoma, octreotide acetate and other long-acting somatostatin formulations are currently the therapeutic principle of first choice to control hormone-mediated symptoms. The consequences of gastric acid hypersecretion in patients with Zollinger-Ellison syndrome are best controlled by proton pump inhibitors. Available data on growth control indicate that stabilization of tumour growth seems to be the most beneficial antiproliferative effect occurring in up to 50% of patients. This effect is limited. However, it is unknown which tumour entity responds best to long- acting somatostatin analogues. Conclusion: Additional studies in patients with known spontaneous tumour growth and avoiding a mix-up of different entities of neuroendocrine malignancies are necessary to identify subpopulations of neuroendocrine tumours which respond to long-acting somatostatin analogues in terms of longer lasting growth inhibition.

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“…Several studies have shown promising results in improved symptoms and longevity in patients with carcinoid syndrome. [12][13][14][15][16] However, for patients with cardiac involvement, worsening right-sided heart failure remains a major cause of morbidity and mortality.…”

Section: Discussionmentioning

confidence: 99%

Carcinoid Heart Disease and Review of Literature

Fernandes¹,

Sattiraju²,

Mehta³

et al. 2017

Heart Res Open J

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Carcinoid Heart Disease (CHD) is one of the rare causes of acquired valvular heart disease. CHD develops as a systemic manifestation of metastatic neuroendocrine tumors (NETs). When these tumors metastasize to the liver, they can release vasoactive amines into the systemic circulation, which can result in carcinoid syndrome and CHD. The key for diagnosing CHD is a thorough history and physical examination supplemented with characteristic echocardiographic findings. We report a case with classic echocardiographic features of CHD, which unfolded the diagnosis of metastatic carcinoid tumor.

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Treatment of the Malignant Carcinoid Syndrome

Cited by 758 publications

References 10 publications

Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours

Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours

Treatment of Neuroendocrine GEP Tumours with Somatostatin Analogues

Carcinoid Heart Disease and Review of Literature

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