Incidence and prognosis of patients with small intestinal neuroendocrine tumors in a population based nationwide study

Snorradottir, S; Asgeirsdottir, A. E.; Rögnvaldsson, Sæmundur; Jónasson, Jón Gunnlaugur; Bjõrnsson, Einar

doi:10.1016/j.canep.2022.102197

Cited by 7 publications

(13 citation statements)

References 17 publications

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“…Studies have shown a steadily rising age-adjusted incidence rate in small intestinal NETs. The highest incidence was in the population between 70 and 79 years, and the lowest was between 20 and 29 years; however, no cases were reported <20 years of age [ 4 , 9 , 10 ]. The patient in this case presented at the age of 18 years with symptoms of abdominal pain and jaundice suggestive of biliary obstruction.…”

Section: Discussionmentioning

confidence: 99%

“…The type of surgery has a significant prognostic implication. Elective surgery carries a better outcome compared with acute surgery [ 9 ]. Open surgery is recommended as laparoscopic procedures are challenged by the presence of small and multifocal lesions [ 20 ].…”

Section: Discussionmentioning

confidence: 99%

“…Here, we present a case of small intestinal NET occurring at a very young age. To the best of the authors’ knowledge, this is the first case of NET of the ampulla of Vater with lymph node metastases reported in a patient <20 years of age [ 4 , 9 ]. This case highlights the importance of a multidisciplinary approach in the diagnosis and management of NETs and demonstrates the efficacy of radical surgical treatment on these tumors.…”

Section: Introductionmentioning

confidence: 99%

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Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report

Alsalman,

Alquorain,

Alyousef

et al. 2024

Case Rep Gastroenterol

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Introduction: Ampullary neuroendocrine tumors (NETs) are usually diagnosed in the 5th–6th decades of life, and no cases were reported in <20 years of age. We report a rare case, presenting at a very young age, of well-differentiated NET involving the ampulla of Vater with lymph node metastasis. Case Presentation: An 18-year-old man presented with a 3-month history of upper abdominal pain and jaundice. Abdominal ultrasound showed a dilated common bile duct, and endoscopic retrograde cholangiopancreatography revealed two duodenal polypoid lesions, one of them overlying the ampulla of Vater, with an erythematous and ulcerated surface. Histopathological examination confirmed the diagnosis of NET grade 1. Octreotide scan revealed 2 para-aortic lymph nodes with intense radiotracer uptake. The patient had undergone Whipple surgery with para-aortic lymph node dissection. Histopathological examination of the surgical specimens was confirmatory of NET grade 2 and paraganglioma in a few of the dissected lymph nodes. Postoperatively, the patient was kept on monthly intramuscular octreotide. Follow-up gallium-68 DOTATATE is unremarkable apart from an avid left para-aortic lymph node which is showing stability over 12 months of follow-up. Conclusion: This case demonstrates that NETs of the ampulla of Vater can present at a very young age. Radical surgical excision with extended lymph node dissection and postoperative octreotide is associated with better patient outcomes and survival.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report

Alsalman,

Alquorain,

Alyousef

et al. 2024

Case Rep Gastroenterol

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“…Although more studies are required, patients with advanced small bowel NET should be carefully considered for surgery, as this has been shown to improve survival [105,[118][119][120]. Furthermore, emergency surgery in patients with small bowel NET is associated with a worse short-term risk of mortality compared with those who undergo elective surgery [121].…”

Section: Prognosis Of Small Bowel Neuroendocrine Tumoursmentioning

confidence: 99%

Small bowel neuroendocrine tumours – casting the net wide

Zammit

Sidhu

2023

Current Opinion in Gastroenterology

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Purpose of review Our aim is to provide an overview of small bowel neuroendocrine tumours (NETs), clinical presentation, diagnosis algorithm and management options. We also highlight the latest evidence on management and suggest areas for future research. Recent findings Dodecanetetraacetic acid (DOTATATE) scan can detect NETs with an improved sensitivity than when compared with an Octreotide scan. It is complimentary to small bowel endoscopy that provides mucosal views and allows the delineation of small lesions undetectable on imaging. Surgical resection is the best management modality even in metastatic disease. Prognosis can be improved with the administration of somatostatin analogues and Evarolimus as second-line therapies. Summary NETs are heterogenous tumours affecting most commonly the distal small bowel as single or multiple lesions. Their secretary behaviour can lead to symptoms, most commonly diarrhoea and weight loss. Metastases to the liver are associated with carcinoid syndrome.

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“…Pivotal studies have either excluded Si-NET G2 or grouped them together with the much more frequent G1 tumours, often with NET of other origin. Within the Si-NET group, G1 tumours are three times as frequent as G2 tumours ( Snorradottir et al 2022 ). Few retrospective series focus on Si-NET, and only one presents solely G2 tumours ( Papantoniou et al 2021 ).…”

Section: Introductionmentioning

confidence: 99%

Treatment efficacy in a metastatic small intestinal neuroendocrine tumour grade 2 cohort

Papantoniou

Grönberg

Thiis‐Evensen

et al. 2023

Endocrine-Related Cancer

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Small intestinal neuroendocrine tumours (Si-NET) are often studied as a uniform group. Proliferation index Ki-67 influences prognosis and determines tumour grade. We hypothesized that Si-NET grade 2 (G2), which have a higher Ki-67 than G1 tumours, might benefit less from established treatments for metastatic disease. We conducted a retrospective cohort study of 212 patients with metastatic Si-NET G2 treated in two Swedish hospitals during 20 years (2000-2019). Median cancer-specific survival (CSS) on first-line somatostatin analogues (SSA) was 77 months. Median progression-free survival (PFS) was 12.4 months when SSA was given as monotherapy, and 19 months for all patients receiving first-line SSA. PFS after SSA dose escalation was 6 months in patients with radiological progression. Treatment efficacies of SSA and peptide-receptor radionuclide treatment (PRRT) were studied separately in patients with Ki-67 of 3-5%, 5-10% and 10-20%. For SSA, PFS was significantly shorter at higher Ki-67 levels (31, 18 and 10 months, respectively) while there was only a minor difference in PFS for PRRT (29, 25 and 25 months). Median PFS for sequential treatment with interferon-alpha (IFNa), everolimus and chemotherapy was 6, 5 and 9 months. IFNa seemed to be effective in tumours with low somatostatin-receptor expression. In conclusion, established treatments appeared effective in Si-NET G2, despite their higher proliferation index compared to G1 tumours. However, efficacy of SSA but not PRRT was reduced at higher Ki-67 levels. SSA dose escalation provided limited disease stabilization.

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Incidence and prognosis of patients with small intestinal neuroendocrine tumors in a population based nationwide study

Cited by 7 publications

References 17 publications

Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report

Obstructive Jaundice Caused by Metastatic Neuroendocrine Tumor of the Ampulla of Vater in a Young Adult: A Case Report

Small bowel neuroendocrine tumours – casting the net wide

Treatment efficacy in a metastatic small intestinal neuroendocrine tumour grade 2 cohort

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