Frequency and Causes of False-Positive Elevated Plasma Concentrations of Fasting Gut Hormones in a Specialist Neuroendocrine Tumor Center

Butler, Olivia; Mekhael, Monica; Ahmed, Adeel; Cuthbertson, Daniel J; Pritchard, David M.

doi:10.3389/fendo.2020.606264

Cited by 6 publications

(4 citation statements)

References 52 publications

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“…CgA is found in most neuroendocrine tumors, and also in local PPGLs, but in the latter this test is inferior to metanephrines, although recommended by some [ 52 ]. Rather, falsely elevated levels may be observed in patients treated with proton pump inhibitors, or in conditions such as atrophic gastritis and inflammatory bowel disease [ 53 ]. Thus, positive results in such settings should be critically interpreted.…”

Section: Resultsmentioning

confidence: 99%

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Calissendorff

Juhlin

Bancos

et al. 2022

Cancers

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Pheochromocytomas and abdominal paragangliomas (PPGLs) are rare tumors arising from the adrenal medulla or the sympathetic nervous system. This review presents a practical guidance for clinicians dealing with PPGLs. The incidence of PPGLs has risen. Most cases are detected via imaging and less present with symptoms of catecholamine excess. Most PPGLs secrete catecholamines, with diffuse symptoms. Diagnosis is made by imaging and tests of catecholamines. Localized disease can be cured by surgery. PPGLs are the most heritable of all human tumors, and germline variants are found in approximately 30–50% of cases. Such variants can give information regarding the risk of developing recurrence or metastases as well as the risk of developing other tumors and may identify relatives at risk for disease. All PPGLs harbor malignant potential, and current histological and immunohistochemical algorithms can aid in the identification of indolent vs. aggressive tumors. While most patients with metastatic PPGL have slowly progressive disease, a proportion of patients present with an aggressive course, highlighting the need for more effective therapies in these cases. We conclude that PPGLs are rare but increasing in incidence and management should be guided by a multidisciplinary team.

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Section: Resultsmentioning

confidence: 99%

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Calissendorff

Juhlin

Bancos

et al. 2022

Cancers

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“…Clearly, VIPoma diagnosis does rely on raised serum levels of VIP. Clinicians should, however, note that false-positive serum gut hormone profiles are possible and repeat levels, taken after strict fasting and interpreted with appropriate radiological imaging, are advisable in the first instance ( 14 ). A mixed secretory component is not uncommon in pNETs and elevated levels of other substances, including PP, may be seen ( 3 , 13 ).…”

Section: Discussionmentioning

confidence: 99%

Metastatic mixed VIPoma/PPoma-induced diarrhoea causing renal failure

Brown

Monaghan

Fristedt

et al. 2022

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Vasoactive intestinal peptide-secreting tumours (VIPomas) are an extremely rare form of functional pancreatic neuroendocrine tumour with an estimated annual incidence of 1 in 10 million. Associated tumour hypersecretion of other peptides, including pancreatic polypeptide (PPomas), may also be seen. These malignancies classically present with a defined triad of refractory diarrhoea, hypokalaemia and metabolic acidosis known as Verner–Morrison syndrome. Diagnosis is frequently delayed, and the majority of patients will have metastatic disease at presentation. Symptoms are usually well controlled with somatostatin analogue administration. Here we report a case of metastatic mixed VIPoma/PPoma-induced diarrhoea causing renal failure so severe that ultrafiltration was required to recover adequate renal function. Learning points Profuse, watery diarrhoea is a common presenting complaint with a multitude of aetiologies. This, combined with the rarity of these tumours, makes diagnosis difficult and frequently delayed. A functional neuroendocrine tumour should be suspected when diarrhoea is unusually extreme, prolonged and common causes have been promptly excluded. These patients are likely to be profoundly unwell on presentation. They are extremely hypovolaemic with dangerous electrolyte and metabolic abnormalities. Aggressive initial rehydration and electrolyte replacement are imperative. A somatostatin analogue should be commenced as soon as the diagnosis is suspected. This is an extreme example of Verner–Morrison syndrome. We are unaware of another case where renal failure secondary to diarrhoea and dehydration was so severe that renal replacement therapy was required to restore adequate renal function, further emphasising how critically unwell these patients can be. Both the primary tumour and metastases showed a remarkably good and rapid response to somatostatin analogue administration. Cystic change and involution were noted on repeat imaging within days. Prior to his illness, this patient was extremely high functioning with no medical history. His diagnosis was an enormous psychological shock, and the consideration and care for his psychological well-being were a crucial part of his overall management. It highlights the importance of a holistic approach to cancer care and the role of the clinical nurse specialist within the cancer multidisciplinary team.

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“…Because gastrin is released by G cells in response to the stimulation of gastrin-releasing peptides in the stomach, and a person's food intake or prolonged use of proton pump inhibitors can lead to fluctuations in serum gastrin levels, the results of the serum gastrin test should be interpreted with caution. [24][25][26] Even if the gastrin levels and atrophy are assessed, several patients do not meet the criteria for ECL NET subtyping. Therefore, applying the conventional ECL-cell NET subtyping criteria is often ambiguous.…”

Section: Clinicopathologic Features Of Micronetsmentioning

confidence: 99%

Gastric Neuroendocrine Tumors According to the 2019 World Health Organization Grading System: A Single-Center, Retrospective Study

et al. 2023

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Background/Aims: Although gastric neuroendocrine tumors (NETs) are uncommon neoplasms, their prevalence is increasing. The clinical importance of the World Health Organization (WHO) classification of gastric NETs, compared with NETs in other organs, has been underestimated. This study aimed to systematically evaluate the clinical and pathologic characteristics of gastric NETs based on the 2019 WHO classification and to assess the survival outcomes of patients from a single-center with a long-term follow-up. Methods:The medical records of 427 patients with gastric NETs who underwent endoscopic or surgical resection between January 2000 and March 2020 were retrospectively reviewed. All specimens were reclassified according to the 2019 WHO classification. The clinicopathologic characteristics, treatment, and oncologic outcomes of 139 gastric NETs were analyzed. Results:The patients' median age was 53.0 years (interquartile range [IQR], 46.0 to 63.0 years). The median follow-up period was 36.0 months (IQR, 15.0 to 63.0 months). Of the patients, 92, 44, and 3 had grades 1, 2, and 3 NETs, respectively. The mean tumor size significantly increased as the tumor grade increased (p=0.025). Patients with grades 2 and 3 gastric NETs more frequently had lymphovascular invasion (29.8% vs 10.9%, p=0.005) and deeper tissue invasion (8.5% vs 0%, p=0.012) than those with grade 1 tumors. The overall disease-specific survival rate was 100%. Two patients with grades 2-3 gastric NETs experienced extragastric recurrence. Conclusions:Although gastric NETs have an excellent prognosis, grade 2 or grade 3 gastric NETs are associated with a larger size, deeper invasion, and extragastric recurrence, which require active treatment.

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Frequency and Causes of False-Positive Elevated Plasma Concentrations of Fasting Gut Hormones in a Specialist Neuroendocrine Tumor Center

Cited by 6 publications

References 52 publications

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Pheochromocytomas and Abdominal Paragangliomas: A Practical Guidance

Metastatic mixed VIPoma/PPoma-induced diarrhoea causing renal failure

Gastric Neuroendocrine Tumors According to the 2019 World Health Organization Grading System: A Single-Center, Retrospective Study

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