Principles of diagnosis and management of neuroendocrine tumours

Raphael, Michael J.; Chan, David; Law, Calvin; Singh, Simron

doi:10.1503/cmaj.160771

Cited by 88 publications

(78 citation statements)

References 67 publications

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“…The present database does not include information regarding patients’ state prior to diagnosis; nonetheless, a previous presence of obesity and metabolic syndrome has been linked with NETs incidence which would require further research [16]. Altogether, comorbidities have a direct influence in the therapeutic choice [2] and should be considered in the principal medical protocols.…”

Section: Discussionmentioning

confidence: 99%

“…Neuroendocrine tumours (NETs) are a type of neoplasms characterised by its capacity to synthesise and secrete monoamines, due to its neuroendocrine origin [1]. NETs are classified according to various features centred on their biological behaviour: grade, differentiation and stage, and by their location [2]. The body-wide distribution of NETs is explained by the extensive presence of neuroendocrine tissue in the human body.…”

Section: Introductionmentioning

confidence: 99%

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Exploring the current status of neuroendocrine tumours: a population-based analysis of epidemiology, management and use of resources

Darbà

Marsà

2019

BMC Cancer

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BackgroundNeuroendocrine tumours (NETs) are rare malignancies characterised by its capacity to synthesise and secrete monoamines, due to its neuroendocrine origin. Its varied locations and symptoms have traditionally been responsible for extended delays in their diagnosis. The interest of this study was to characterise the patient population diagnosed with NETs in Spain and to revise how the disease is managed, together with the hospitalisation costs of these patients.MethodsThe database included records of all patients diagnosed with a NET between 2010 and 2015. Admission records were used to evaluate hospitalisation, disease management data and costs, and single-patient files were used to characterise the population.ResultsNine Thousand One Hundred Twenty patients were diagnosed with a neuroendocrine tumour between 2010 and 2015, with a 2 fold increase in the diagnosis rate over the study period. 42.25% of the patients were females, while 57.75% were males, and mean diagnosis age was 62.58 years (SD = 14.65). Considering all the registered neuroendocrine neoplasms, 46.86% of the patients had malignant well-differentiated NETs, 32.02% had a malignant poorly differentiated neuroendocrine carcinoma and 42.93% of patients developed metastatic NETs. In addition, 18.59% of patients were diagnosed with benign well-differentiated NETs. The most common tumour sites were the bronchus, lung and other sites, including pancreatic tumours; metastasis was found in the liver and distant lymph nodes. Pancreatic resection was the most common surgical procedure utilised in these patients, summing 19% of total expenses, the injection of an unspecified therapeutic substance (including targeted therapies) was registered in 11.40% of admissions, while chemotherapy was registered in only 6.85% of admissions. The annual healthcare cost of NETs was €15,373,961, corresponding to €9092 per patient.ConclusionsThe implementation of standard diagnosis procedures should be prioritised, with a focus on the pancreas and lung, and taking into account that 42.93% of the patients develop a metastatic tumour. The presence of comorbidities and multimorbidities should be considered in order to develop more efficient disease management protocols.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Exploring the current status of neuroendocrine tumours: a population-based analysis of epidemiology, management and use of resources

Darbà

Marsà

2019

BMC Cancer

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“…Neuroendocrine tumors (NETs) are relatively rare neoplasms that can occur in a wide range of sites, including the gastrointestinal (GI) tract, pancreas, and lung . Although the clinical behavior of these tumors is often heterogenous and unpredictable, in most cases these tumors are slow‐growing and often asymptomatic.…”

Section: Introductionmentioning

confidence: 99%

“…The optimal medical management of asymptomatic, unresectable NETs is controversial , in part because a substantial proportion of patients can remain progression free—even without treatment—for months or even years. For this reason, many authors and clinical guidelines recommend active surveillance in asymptomatic patients with low‐grade, nonfunctional tumors. However, this approach has an important drawback: the unpredictable clinical course of these tumors, which tend to progress, even when the biological characteristics of the tumor are favorable .…”

Section: Introductionmentioning

confidence: 99%

Meta-Analysis of Randomized Clinical Trials Comparing Active Treatment with Placebo in Metastatic Neuroendocrine Tumors

et al. 2019

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Background. Most guidelines still recommend active surveillance for patients with asymptomatic, unresectable neuroendocrine tumors (NETs). However, recent findings from several randomized placebo-controlled trials suggest that most patients would benefit from active treatment. We conducted a metaanalysis of pooled outcomes from clinical trials in which an active treatment arm was compared with placebo to determine whether active treatment provides a survival advantage. Materials and Methods. This meta-analysis evaluated six trials that compared a medication with placebo in patients with an asymptomatic, metastatic NET. The trials were heterogenous with regard to the active medication (octreotide, lanreotide, sunitinib, everolimus, Lu-Dotatate) and tumor localizations (gastrointestinal, pancreas, lung). Overall survival (OS) and progression-free survival (PFS) for the placebo and active treatment arms were obtained from individual trial data and combined to obtain pooled outcomes. Results. The individual trials all reported significantly better PFS outcomes for active treatment. The pooled data confirmed this advantage. At months 3, 6, 12, 18, and 24, pooled PFS rates for the placebo and treatment arms, respectively, were 92.9% versus 96.9%; 54.3% versus 83.7%; 35.5% versus 68.5%; 25.1% versus 54.7%; and 17.7% versus 61.0%. OS was also higher in the active treatment groups. At months 6, 12, 24, 36, 48, and 60, OS rates (placebo vs. active treatment), respectively, were 88.1% versus 93.4%; 84.1% versus 86.2%; 67.4% versus 76%; 56.6% versus 64.4%; 49.9% versus 61.0%; and 41.7% versus 45.9%. Conclusion. This meta-analysis confirms findings from recent clinical trials indicating that active treatment yields better survival outcomes than placebo. Importantly, these findings were obtained across a wide range of patient profiles and diverse medical treatments for metastatic NETs. Given the lack of reliable prognostic factors to determine a priori which patients are unlikely to benefit from active treatment, these findings support early treatment in most patients. The Oncologist 2019;24:e1315-e1320 Implications for Practice: Although most guidelines still recommend active surveillance for patients diagnosed with metastatic neuroendocrine tumors, the results of this meta-analysis, together with recent data from key clinical trials, suggest that most patients could benefit from upfront active treatment. However, more data are needed to confirm this.

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“…Neuroendocrine tumors (NETs) are rare malignancies that originate from the neuroendocrine system. These tumors can secrete monoamines and are categorized based on features pertaining to their biological behavior, including tumor grade, degree of differentiation, and tumor stage [ 1 ]. According to previous reports, these tumors primarily arise in the lungs, pancreas, and gastrointestinal tract, albeit they can arise in several body organs and thereby cause a wide range of symptoms [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Successful resection of a centrally located primary hepatic neuroendocrine tumor

Alakeel

Alshamrani

Alharbi

et al. 2020

International Journal of Surgery Case Reports

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Principles of diagnosis and management of neuroendocrine tumours

Cited by 88 publications

References 67 publications

Exploring the current status of neuroendocrine tumours: a population-based analysis of epidemiology, management and use of resources

Exploring the current status of neuroendocrine tumours: a population-based analysis of epidemiology, management and use of resources

Meta-Analysis of Randomized Clinical Trials Comparing Active Treatment with Placebo in Metastatic Neuroendocrine Tumors

Successful resection of a centrally located primary hepatic neuroendocrine tumor

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