Endocrine Abstracts

Background: Appendiceal neuroendocrine tumours (aNETs) are usually diagnosed incidentally at appendicectomy, are indolent and rarely recur.Methods: We retrospectively analysed patients diagnosed with aNETs and entered prospectively into our tertiary referring centre’s NET database between 1990 and 2016.Results: 102 patients (F:M ratio of 1.04:1) presented with aNET at a median age of 39.4 (range 16.3–81.1) years. Average ...

Neuroendocrine tumours (NETs) occur in multiple tissues and vary widely in clinical behaviour. Up to 70% of diagnosed patients have elevated circulating levels of chromogranin A, the only serum marker commonly used in clinical practice. However, its low sensitivity and the identification of confounding factors that compromise specificity have raised the need for additional or alternative markers for disease detection and management. Here we aimed to discover candidate NET mark...

Long acting somatostatin analogues (SSA) such as Sandostatin LAR and Lanreotide Autogel are the most commonly used drugs in the management of neuroendocrine tumours (NETs) due to their ability to control symptoms and prolong survival. SSAs use is associated with changes in glucose metabolism. However, there is lack of data for such effects in patients with NETs. We evaluated the effects of SSA on BMI and HbA1c in our cohort of patients with NETs.Methods:...

Background: Current ENET guidelines inform our management of GCCs but we still have poor understanding of how these tumours behave in clinical practice.Methods: We retrospectively analysed patients diagnosed with GCCs and entered prospectively into our tertiary referring centre’s NET database between 1990 and 2016.Results: 41 patients (F:M ratio of 1.28:1) presented with GCC at a median age of 61 (range 27–79) years. Aver...

Background: Symptom management is a cornerstone of clinical care, particularly for patients with cancer. However, often patient’s symptoms go undetected by health care providers between clinic visits. Mobile health applications are increasingly used in oncological care to support cancer patients.Aims: To develop a patient focussed mobile application to enable patients to track symptoms, record quality of life, communicate with their clinical teams a...

Background: Hepatic arterial embolisation (HAE) is a recommended treatment option for patients with neuro-endocrine neoplasms (NENs) and liver metastasis. We aimed to describe outcomes after HAE for NENs at a single United Kingdom centre and investigate the influence of tumour characteristics.Methods: Retrospective analysis of sequential patients with NENs undergoing HAE at The Christie NHS Foundation Trust between 2004 and 2018. Primary end points were ...

Introduction: MEN1 associated pNETs share a unique profile in comparison to sporadic pNETs. The literature on pNETs in MEN1 is evolving with on-going ambiguities in presentation and management. We present a case series of MEN1 associated pNETs from a tertiary centre of excellence for NETs in the UK.Methods: Institutional NET database was used for retrospective data analysis for all patients with pNETs associated with MEN1.Results: ...

Introduction: Some functional neuroendocrine tumours (NETs) secrete serotonin, leading to the symptoms of carcinoid syndrome (e.g. diarrhoea and flushing). In this condition, increased urinary concentrations of the serotonin metabolite 5-hydroxyindoleacetic acid (5-HIAA) are often detected. Measurement of 24-hour urinary 5-HIAA is therefore used to investigate patients with confirmed or suspected carcinoid syndrome. We sought to evaluate the appropriateness and effectiveness o...

Introduction: Gastric NENs are increasingly being diagnosed at endoscopy. The majority of these are type 1 gastric NEN. However, around 10–20% can be type 3 or type 4 Gastric NENs.Methods: A retrospective review performed of 54 patients with gastric NENs referred to a tertiary centre during 2014 to 2019.Results: Of the 54 cases referred, 45 were type 1 gastric NENs and the 9 were type 3 Gastric NENs. Of the 45 type 1 gastric N...

Background: Type III gastric neuroendocrine neoplasms (g-NEN) are usually lesions of higher grade and often associated with loco-regional and distant metastases. Guidelines recommend partial or total gastrectomy with local lymph node resection. Data for less-extensive resections are limited.Aim: To assess whether advanced endoscopic excision [Endoscopic Mucosal Resection (EMR) or Endoscopic Submucosal Dissection (ESD)] or localized surgical approach (wed...

Introduction: Somatostatin analogues (SSA) are a cornerstone in the treatment of neuroendocrine tumours. Two clinical trials have defined the disease control effect of these treatments; the PROMID study of Octreotide LAR v’s placebo, median time to tumour progression (TTP) 14.2 m v’s 6.0 m, and the CLARINET study (Lanreotide v’s placebo), median progression free survival (PFS) 31 m v’s 18 m. In every day practice, however, many neuroendocrine tumours commen...

Background: Appendiceal GCAs (previously known as Goblet Cell Carcinoids) are considered to have an unpredictable behaviour; even in node-negative patients, metastases mainly local to the adjacent peritoneum are seen. The systemic chemotherapy (scx) is often borrowed from colon cancer regimens. We designed a retrospective study with the aim of describing the use of scx in these rare malignancies in our institution.Methods: Data from all consecutive cases...

Introduction: Perioperative mortality of patients who undergo heart valve surgery for carcinoid heart valve disease (CHVD) has been observed to be high (5–10%). We investigated whether peptide receptor radiotherapy (PRRT) with Lutetium-177 Dotatate can be used safely in patients with neuroendocrine neoplasms (NEN) and CHVD and if there is an associated survival advantage by reducing overall exposure of the valves to high doses of vasoactive peptides.<p class="abstext"...

Background: Liver embolisation is one of the treatment options available for patients diagnosed with neuro-endocrine neoplasms (NEN), especially in the presence of carcinoid syndrome. It is still uncertain whether the benefits of the various types of embolisation treatments truly outweigh the complications in NENs. This systematic review assesses the available data relating to liver embolisation in patients with NENs.Methods: Eligible studies (identified...

Introduction: Neuroendocrine tumours of the gastrointestinal tract often cause carcinoid syndrome and in advance cases carcinoid heart disease. These tumours secrete serotonin, which can bind to heart valves and cause fibrosis and eventually valve incompetence. Most cases involve the tricuspid valve +/− pulmonary valve. Medical management comprises diuretics for fluid overload and somatostatin analogues to reduce circulating serotonin. Definitive treatment is heart-valve...

We report a retrospective, single centre observational study to determine the characteristics and outcomes of pancreatic insulinoma. Interrogation of the Oxford NET database, a departmental database of new NET MDT referrals since 2011 (n = 1059), revealed 23/1059 (2.1%) patients with insulinoma diagnosed and treated between 2011 and 2018. Patient records were reviewed and data was captured on demographics, presentation, investigations, treatments and outcomes: 15/23 (...

Background: Paragangliomas are rare neuroendocrine tumours arising in the autonomic nervous system, which can secrete excess catecholamines, causing cardiovascular complications. Malignancy occurs in up to 23% of paragangliomas, resulting in a poor prognosis with a 5-year mortality of 43–63%. There’s a limited evidence base for their treatment, with no official guidelines. Peptide Receptor Radionuclide Therapy (PRRT) is a proven treatment for neuroendocrine tumours o...

Introduction: In the UK, the fasting plasma concentrations of a panel of gut hormones (vasoactive intestinal peptide (VIP), gastrin, pancreatic polypeptide, glucagon and somatostatin as well as chromogranin A) are frequently measured during the evaluation of patients who have confirmed or clinically suspected neuroendocrine tumours (NETs). However, elevated concentrations of these hormones are sometimes also detected in patients who have no other evidence of a NET. We sought t...

Background: Gastric and duodenal neuroendocrine tumours are increasing in incidence. ENETS guidelines suggest endoscopic resection of type 1 gastric NENs over 10 mm and consider endoscopic resection for duodenal NENs. However, the endoscopic techniques are not clearly mandated. There, is evidence to suggest endoscopic mucosal dissection has higher incidence of R0 resections compared with polypectomy or EMR alone. We have reviewed our data of endoscopic resection of gastro-duod...

Introduction: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is documented as a pre-malignant condition by the World Health Organisation. Patients with pulmonary nodules typically undergo 5′-18fluoro-deoxy-D-glucose (FDG) positron emission tomography combined with computed tomography (PETCT) for further diagnostic investigation. DIPENCH is widely accepted as an FDG negative pathology. The purpose of this analysis was to a...

Mitotane is an inhibitor of adrenal steroidogenesis with cytostatic activity used in the adjuvant and palliative treatment of adrenocortical cancer. Evidence shows that achieving a therapeutic range of between 14 and 20 mg/l is associated with optimal disease control whereas blood levels above this can result in more severe gastrointestinal and neurological toxicity. Therapeutic drug monitoring is available but in real life achieving and remaining within the ideal range can be...

Introduction: Lutetium-177 Oxodotreotide (Lutathera) is a targeted molecular radiotherapy treatment for advanced, inoperable, progressive neuro-endocrine tumours. We present our experience of Lutathera therapy with particular focus on our experience implementing treatment as a day-case procedure, and assessment of adherence to treatment quality standards surrounding the administration.Aims: To assess safety of Lutathera as a day-case treatment.<p cla...

Neuroendocrine tumours (NETs) are a heterogenous group of tumours that arise from neuroendocrine cells throughout the body. Debulking surgery is offered to patients with metastatic NETs and is recommended in the ENETS guidelines. This study was undertaken to evaluate the impact of debulking surgery and liver directed therapy (TACE, TAE) in metastatic NETs, on quality of life (QoL). Prospective longitudinal cohort study was undertaken at University Hospital Southampton. Patient...

Background: Transformation of the South Wales NET service in late 2017 introduced new specialised staff, gastroenterology-led clinics and MDTs with a particular emphasis on educating referring specialties on NETs. Data has shown patient experience, satisfaction rates, quality of life and gastrointestinal symptom scores have all improved. This study aims to describe findings from new NET service data for: referrals into the service, diagnosis times after symptoms, and initial s...

Neuroendocrine tumours (NETs) are distinctive tumours which arise from cells which have neural and hormonal origins. Gastroenteropancreatic tumours (GEP) are the most common clinically encountered NETs. Imaging has a lead role in the evaluation of these tumours – detection, staging and follow-up. It is also known that PET–CT with 18F-FDG has a limited value in well-differentiated NETs. This study was performed to evaluate the clinical value of 18</sup...

Background: When seeing patients with gastroenteropancreatic neuroendocrine tumours (GEP-NETs) in clinic, we noticed that a significant proportion of our patients also had additional primary malignancies. This observation was also raised by patients at our local NET Patient Foundation support group. The idea that patients with GEP-NETs are more prone to additional malignancies is supported in the literature therefore we wanted to determine the proportion of our patients who ha...

Explosive, multiple episodes of diarrhoea are common in patients with neuroendocrine neoplasms affecting around 30% of the patient population. This is combination with other symptoms such as red, dry flushing and wheezing constitute carcinoid syndrome. This syndrome can have a devastating effect on patients impacting upon all aspects of their being. Somatostatin analogues alone can be successful in the management of diarrhoea however in some cases this is inadequate. In 2018 T...

Introduction: Phaeochromocytoma (PHAEO) and paraganglioma (PGL) are neuroendocrine tumors arising from neural crest-derived cells either in the adrenal gland (PHAEO) or along the central sympathetic and parasympathetic chains (PGL), including the carotid body. There is convincing evidence linking hypoxia pathways with development of PHAEO/PGL especially with genetic susceptibility. Association between PHAEO and PGL and cyanotic congenital heart disease (CHD) are well recognise...

DOTA (1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid)−peptide (DOTA peptide) seen on positron emission tomography combined with computed tomography (68Ga-DOTA PETCT) has been proven to be one of the most accurate methods of imaging NETs. The first stage in the accurate reporting and utilising DOTA PETCT is a clear understanding of the normal patterns. PNETs can present with focal DOTA peptide uptake on imaging. We present preliminary data of a retros...

Background: Patients with low grade GI tract (GEP) neuroendocrine tumours and low grade neuroendocrine tumours of unknown primary (LG NETs) often live for many years even in the context of advanced disease. Their nutritional status is important to maintain wellbeing. Both the tumour and its treatment can potentially affect nutritional status.Method: Patients attending routine follow up appointments with the NET team in Edinburgh were invited to participa...

We present the case of a 54-year-old woman with known metastatic non-functional pancreatic NET since 2004 that developed new debilitating recurrent hypoglycemia in 2013. The patient presented in 2004 with disseminated malignancy. Radiology revealed a large pancreatic mass associated with multiple liver lesions and abdominal lymphadenopathy. Liver biopsy confirmed well differentiated NET. Initial treatment with cisplatin etoposide chemotherapy was poorly tolerated and discontin...

Cardiac paraganglioma is one of the most infrequent primary cardiac tumours, with <50 cases reported in the literature. Left atrium is the most common location. This is a case of a 57-year-old gentleman who presented with 7 year history of gradually increasing cranial perspiration, irritability, anxiety, unexplained feelings of fear, facial erythema, hypertension and atrial fibrillation. Following investigations of atrial fibrillation, a cardiac MRI scan revealed a mass be...

We present a 66 year old man with a metastatic small bowel neuroendocrine tumour managed with resection of primary in 2011 and subsequent somatostatin analogue therapy. In 2018 disease progressed requiring right ureteric stenting due to extrinsic nodal compression. Three weeks later he presented with sweats, pyrexia and elevated inflammatory markers. Multiple blood and urinary cultures were clear. CT imaging, MRI spine and echocardiogram did not identify a source of sepsis. Ur...

Neuroendocrine tumours presenting in the bronchial or gastrointestinal tract often present with metastatic disease. Whereas well recognised sites of metastases include liver, lymph nodes and bones, they can also arise in unusual locations sometimes causing difficulties in diagnosis. The breast is an uncommon site for neuroendocrine tumour metastasis with estimates that it represents 0.1% of all breast tumours. Due to the low incidence of these neoplasms and due to difficulties...

Aim: The purpose of this literature review aims to critically analyse how exercise affects quality of life (QoL) in cancer patients. The initial idea for this review was to focus on exercise and neuroendocrine tumours (NETs). As there is lack of evidence in this field, it meant a greater and widened search lead to focusing on solid tumour types as they are the most similar to NETs.Method: Methodology used to conduct this review was by using the Preferred...

Von Hippel-Lindau (VHL) disease is an inherited tumour syndrome, caused by a mutation in the VHL tumour suppressor gene encoding the VHL protein. Patients are prone to cysts and neuroendocrine tumours in the pancreas and other benign and malignant neoplasms. Pancreatic cysts occur in approximately 70% of VHL patients. We describe two cases of VHL disease with extensive multi-cystic changes affecting the whole pancreas, both patients had deletions of exon 2–3 of the VHL ge...

Patients with neuroendocrine disease now have the option of having peptide receptor radionuclide therapy (PRRT) for both symptomatic treatment and reducing disease progression. Lutetium (177Lu) oxodotreotide is funded centrally in the UK and several sites have recently begun to provide the treatment for their patients. There are, however, several logistical, operational and regulatory obstacles which must be navigated to ensure the service is both safe and efficient...