Neuroendocrine Tumours (NETS): Telemedicine and patient satisfaction in the COVID-19 pandemic: A patient survey from a European Neuroendocrine Tumour Centre of Excellence

Background & Aims: Chromogranin A (CgA) is the most important general tumour marker used in the diagnosis and follow-up of patients with neuroendocrine tumours (NET). Chromogranin A assays may have different sensitivities, which is of importance for the clinical diagnosis and handling of NET patients. The aim of this study was to compare the clinical sensitivities of three different CgA assays in NET patients.Methods: We measured CgA level in 42 NET patients (male/female: 23/19, median age: 63 years, range 29- 85 years). Twenty-five patients had liver metastases, eight had local disease, and nine were disease free after surgery. We studied an in-house RIA: RH RIA assay (Rigshospitalet, Copenhagen, Denmark); NEOLISATM (Euro Diagnostica, Malmö, Sweden) and EURIA CgA RIA (Euro Diagnostica, Malmö, Sweden).Results: The RH RIA assay showed a clinical sensitivity of 97%, while the NEOLISA and EURIA assays both showed similar clinical sensitivities of 79%. Patients with liver metastases had significantly higher CgA levels compared to disease free patients by all three assays (P<0.001), but only the RH RIA assay was able to discriminate between patients with liver metastases and with regional disease (P<0.01).Conclusion: Chromogranin A measurements are significantly assay-dependent and caution should be applied in the interpretation of CgA measurement for assessment of NET status. The in-house RH RIA assay was better at predicting NET status than the NEOLISA and EURIA assays.List of abbreviations: CgA: chromogranin A; EURIA: Eurodiagnostica, radioimmunoassay; GFR: glomerular filtration rate; NEOLISA: Eurodiagnostica, enzyme-linked immunosorbent assay; NET: neuroendocrine tumour; PET-CT: positron emission tomography; PPI: proton pump inhibitor; RH RIA: Rigshospitalet, radioimmunoassay ; SPECT-CT: single photon emission computed tomography.

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Positivity for SATB2 distinguishes Islet1 positive rectal neuroendocrine tumours from pancreaticoduodenal neuroendocrine tumours

Journal of Clinical Pathology ◽

10.1136/jclinpath-2020-206645 ◽

2020 ◽

pp. jclinpath-2020-206645

Author(s):

Sambit Kumar Mohanty ◽

Ankit Tiwari ◽

Nitin Bhardwaj ◽

Fai Chuang ◽

Evelyn Kim ◽

...

Keyword(s):

Predictive Value ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Tissue Microarrays ◽

Characteristic Analysis ◽

Therapeutic Implications ◽

Weak Staining ◽

Metastatic Setting ◽

Strong Staining ◽

Paraffin Tissue

AimsDetermining the site of origin of a metastatic neuroendocrine tumour (NET) can be challenging and has important prognostic and therapeutic implications. An immunohistochemical (IHC) panel consisting of TTF1, CDX2, PAX8/PAX6 and Islet1 is often employed. However, there can be a significant IHC overlap among different primary sites. Herein, we sought to determine the utility of including Special AT-rich sequence binding protein-2 (SATB2) in the IHC panel that is used for determining the site of origin of a metastatic NET.MethodsParaffin tissue microarrays consisting of 137 primary NETs (26 lung, 22 jejunoileal, 8 appendix, 5 stomach, 4 duodenum, 17 rectum and 55 pancreas) were stained for SATB2, in addition to the well-described lineage-associated markers, such as TTF1, CDX2, PAX6 and Islet1. Additionally, a tissue microarray consisting of 21 metastatic NETs (1 lung, 1 stomach, 8 jejunoileal and 11 pancreas) was stained for TTF1, CDX2, SATB2 and Islet1. The results were recorded as no staining, weak staining and moderate to strong staining.ResultsAll appendiceal NETs and majority (88%) of the rectal NETs were positive for SATB2. All primary foregut NETs (stomach, pancreas, duodenum and lung) were negative for SATB2, except for one pulmonary NET with weak staining. However, among the metastatic tumours, 5 of 11 pancreatic NETs, 1 stomach NET, 1 lung NET and 2 of 8 jejunoileal NETs showed weak staining. Receiver operating characteristic analysis incorporating sensitivity and specificity data of IHC panel, considering moderate to strong staining as truly positive cases, showed that inclusion of SATB2 to the previously described NET IHC panel outperformed the panel without SATB2, raising the specificity for pancreaticoduodenal NETs from 81.2% to 100%, with a positive predictive value (PPV) of 100% and negative predictive value (NPV) of 82.22% (p<0.0001); for appendiceal NETs the specificity changed from 99.1% to 98.5% and sensitivity increased from 11.8% to 80%, with a PPV and NPV of 66.67% and 99.26%, respectively (p<0.0001); and for rectal NETs the specificity increased from 97.6% to 99.3% and sensitivity raised from 7.1% to 66.7%, with a PPV and NPV of 80% and 98.53%, respectively (p<0.0001).ConclusionsSATB2 stain is useful in differentiatingIslet1/PAX6 positive pancreatic and rectal NETs, as rectal NETs are typically moderately to strongly positive for SATB2 and pancreatic NETs are usually negative or weakly positive for SATB2. Moderate to strong staining for SATB2 is suggestive of an appendiceal or a rectal primary. SATB2 may complement the panel of CDX2, TTF1 and Islet1 in determining the site of origin of an NET in a metastatic setting.

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Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms

Endocrine Related Cancer ◽

10.1530/erc-11-0013 ◽

2011 ◽

Vol 18 (S1) ◽

pp. S1-S16 ◽

Cited By ~ 180

Author(s):

Günter Klöppel

Keyword(s):

Gastrointestinal Tract ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Tnm Stage ◽

Neuroendocrine Neoplasms ◽

Gastroenteropancreatic Neuroendocrine Neoplasms ◽

Poorly Differentiated ◽

Well Differentiated ◽

Neuroendocrine Carcinomas

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are composed of cells with a neuroendocrine phenotype. The old and the new WHO classifications distinguish between well-differentiated and poorly differentiated neoplasms. All well-differentiated neoplasms, regardless of whether they behave benignly or develop metastases, will be called neuroendocrine tumours (NETs), and graded G1 (Ki67 <2%) or G2 (Ki67 2–20%). All poorly differentiated neoplasms will be termed neuroendocrine carcinomas (NECs) and graded G3 (Ki67 >20%). To stratify the GEP-NETs and GEP-NECs regarding their prognosis, they are now further classified according to TNM-stage systems that were recently proposed by the European Neuroendocrine Tumour Society (ENETS) and the AJCC/UICC. In the light of these criteria the pathology and biology of the various NETs and NECs of the gastrointestinal tract (including the oesophagus) and the pancreas are reviewed.

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Nurse developments in neuroendocrine tumour management.

Endocrine Related Cancer ◽

10.1677/erc.0.0100487 ◽

2003 ◽

pp. 487-488 ◽

Cited By ~ 3

Author(s):

C Bouvier

Keyword(s):

Multidisciplinary Team ◽

Significant Contribution ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Nurse Specialist ◽

Royal Free Hospital ◽

The Past ◽

Standards Of Practice ◽

High Standards ◽

Set Up

Nurse developments in the management of neuroendocrine tumours have changed significantly over the past three years. At the Royal Free Hospital we set up the nurse specialist role due to the expansion of patients being referred to the specialist unit, and the obvious need for a nursing input into their care. The nurse specialist can make a significant contribution within the context of a multidisciplinary team especially in the production of guidelines and policies to ensure and maintain high standards of practice, education for the patient, and the provision of expertise and security that the patient requires when diagnosed with a rare disease.

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Neuroendocrine Tumour of the Appendix in Pregnancy

Acta Chirurgica Latviensis ◽

10.2478/chilat-2014-0017 ◽

2013 ◽

Vol 13 (2) ◽

pp. 81-83

Author(s):

Andrejs Vanags ◽

Ilze Strumfa ◽

Zane Simtniece ◽

Jānis Gardovskis

Keyword(s):

Acute Appendicitis ◽

Digestive System ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Pathological Examination ◽

Solitary Lesion ◽

Documented Case ◽

Pubmed Database ◽

Anatomic Localization ◽

In Pregnancy

Abstract Despite the fact that the appendix is one of the rarest anatomic localization of neuroendocrine tumours (NETs) in the digestive system, the appendicular neuroendocrine tumour (A-NET) remains among the most common tumours of the appendix. The A-NET usually presents as a small solitary lesion revealed only after pathological examination of appendix that has been surgically removed for acute appendicitis. Only 18 papers in the Pubmed database (1965-2012) are dedicated to A-NET in pregnancy. Here we present a well-documented case of A-NET in a pregnant lady.

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Doctors’ trustworthiness, practice orientation, performance and patient satisfaction

International Journal of Health Care Quality Assurance ◽

10.1108/ijhcqa-04-2013-0037 ◽

2015 ◽

Vol 28 (1) ◽

pp. 82-95 ◽

Cited By ~ 8

Author(s):

Barend Van Den Assem ◽

Victor Dulewicz

Keyword(s):

Patient Satisfaction ◽

Patient Survey ◽

The Other ◽

Professional Relationships ◽

Hierarchical Regression ◽

Patient Relationship ◽

Doctor Patient Relationship ◽

Content Type ◽

New Model ◽

And Performance

Purpose – The purpose of this paper is to provide a greater understanding of the general practitioner (GP)-patient relationship for academics and practitioners. A new model for dyadic professional relationships specifically designed for research into the doctor-patient relationship was developed and tested. Various conceptual models of trust and related constructs in the literature were considered and assessed for their relevance as were various related scales. Design/methodology/approach – The model was designed and tested using purposefully designed scales measuring doctors’ trustworthiness, practice orientation performance and patient satisfaction. A quantitative survey used closed-ended questions and 372 patients responded from seven GP practices. The sample closely reflected the profile of the patients who responded to the DoH/NHS GP Patient Survey for England, 2010. Findings – Hierarchical regression and partial least squares both accounted for 74 per cent of the variance in “overall patient satisfaction”, the dependent variable. Trust accounted for 39 per cent of the variance explained, with the other independent variables accounting for the other 35 per cent. ANOVA showed good model fit. Practical implications – The findings on the factors which affect patient satisfaction and the doctor-patient relationship have direct implications for GPs and other health professionals. They are of particular relevance at a time of health reform and change. Originality/value – The paper provides: a new model of the doctor-patient relationship and specifically designed scales to test it; a greater understanding of the effects of doctors’ trustworthiness, practice orientation and performance on patient satisfaction; and a new framework for examining the breadth and meaning of the doctor-patient relationship and the management of care from the patient’s viewpoint.

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Is patient satisfaction in primary care dependent on structural and organizational characteristics among providers? Findings based on data from the national patient survey in Sweden

Health Economics Policy and Law ◽

10.1017/s1744133112000333 ◽

2012 ◽

Vol 8 (3) ◽

pp. 317-333 ◽

Cited By ~ 12

Author(s):

Anna H. Glenngård

Keyword(s):

Primary Care ◽

Patient Satisfaction ◽

Social Deprivation ◽

Primary Care Providers ◽

Patient Survey ◽

Structural Factors ◽

Care Providers ◽

Factors Affecting ◽

Provider Performance ◽

National Patient

AbstractIn parallel to market-like reforms in Swedish primary care, the gathering and compilation of comparative information about providers, for example through survey tools, has been improved. Such information is increasingly being used to guide individuals’ choice of provider and payers’ assessments of provider performance, often without critically reflecting about underlying factors affecting the results. The purpose of this study was to analyze variation in patient satisfaction, with respect to organizational and structural factors, including the mix of registered individuals, among primary care providers, based on information from a national patient survey in primary care and register data in three Swedish county councils. Systematic variation in patient satisfaction was found with respect to both organizational and structural factors, including characteristics of registered individuals. Smaller practices and practices where a high proportion of all visits were with a doctor were associated with higher patient satisfaction. Also practices where registered individuals had a low level of social deprivation and a high overall illness on average were associated with higher patient satisfaction. Factors that are of relevance for how well providers perform according to patient surveys are more or less possible to control for providers. This adds to the complexity for the use of such information by individuals and payers to assess provider performance.

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MON-903 Sporadic Phaeochromocytoma, Pancreatic Neuroendocrine Tumour and a Sacral Hibernoma: A Case Report

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1396 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Ravind Singh Pandher ◽

Emily Hibbert

Keyword(s):

Adrenal Gland ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Case Reports ◽

Genetic Mutation ◽

Ki 67 ◽

Tissue Mass ◽

Pancreatic Tail ◽

Pancreatic Neuroendocrine Tumour ◽

The Right

Abstract Most phaeochromocytomas and pancreatic neuroendocrine tumours are sporadic in nature however the presence of multiple neuroendocrine tumours raises the suspicion of a hereditary endocrinopathy. Hibernomas, benign tumours that morphologically resemble brown fat, do not possess a clear aetiology and a link with other neuroendocrine tumours remains unclear. We report an unusual case of a concurrent sporadic phaeochromocytoma, pancreatic neuroendocrine tumour and a sacral hibernoma. A 61 year old female presented with a 3 month history of abdominal pain which led to the discovery of a lesion in her right adrenal gland and a soft tissue mass at the pancreatic tail on a CT Abdomen. The adrenal lesion was biochemically suggestive of a phaeochromocytoma (plasma normetanephrine 4930 pmol/L, plasma 3-methoxytyramine 580 pmol/L, urinary noradrenaline 5564 pmol/day, urinary dopamine 4720 nmol/day). A 68Ga-DOTATATE-PET-CT scan revealed DOTATATE avid lesions in the right adrenal gland, tail of pancreas and right sacral ala. Following preoperative medical therapy, the patient underwent a right adrenalectomy and a resection of the distal pancreatic lesion.Histopathology confirmed a phaeochromocytoma with no conscipicouous mitotic activity, and the pancreatic tail lesion was consistent with a well-differentiated neuroendocrine tumour (NET) (Ki-67 score <3%). Following normalisation of the serum catecholamines, a biopsy of the sacral lesion was undertaken, which returned positive for a hibernoma. Genetic testing revealed no identifiable genetic mutations.This case reports the synchronous presence of a phaeochromocytoma, pancreatic NET and sacral hibernoma with no identifiable genetic mutation. To date, the association between hibernomas and neuroendocrine tumours has not been fully established, but a few case reports suggest a possible association between MEN1 and hibernomas.

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Neuroendocrine tumour in a patient with neurofibromatosis type 1 and HIV

Southern African Journal of HIV Medicine ◽

10.4102/sajhivmed.v16i1.323 ◽

2015 ◽

Vol 16 (1) ◽

Author(s):

Juliane Hiesgen ◽

Ebrahim Variava

Keyword(s):

Diabetes Mellitus ◽

Peptic Ulcer ◽

Antiretroviral Therapy ◽

Neurofibromatosis Type 1 ◽

Neuroendocrine Tumours ◽

Neuroendocrine Tumour ◽

Neurofibromatosis Type ◽

Hiv Positive ◽

Ulcer Disease

We report the case of an HIV-positive female patient with neurofibromatosis type 1 who wastreated for recurrent peptic ulcer disease and later developed diabetes mellitus and chronicdiarrhoea. A metastasising somatostatinoma was histologically proven and evidence of aconcomitant gastrin-producing neuroendocrine tumour was found. Neuroendocrine tumours (NETs) are very rare neoplasms originating from a wide variety of endocrine and nervoussystem tissue with the ability to produce different hormones. A somatostatin- and gastrinsecreting NET in a patient with HIV has not been reported in the literature, to the best ofour knowledge. We discuss oncogenic pathomechanisms related to the underlying conditionsand propose stringent monitoring for tumours in HIV-positive patients with phakomatoses aswell as initiation of antiretroviral therapy.

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