Endocrine Abstracts

Background: Neuroendocrine tumours (NETs) represent a diverse group of neoplasms arising from neuroendocrine cells. Streptozotocin (STZ) based chemotherapy regimens have been utilized in the treatment of NETs, yet comprehensive clinical outcomes remain to be fully elucidated.Methods: We retrospectively analyzed median overall (mOS) & progression-free survival (mPFS) among 26 patients with pancreatic and non-pancreatic NETs and NECs treated with STZ-b...

Introduction: Islet cells within the pancreas regulate blood glucose through the production of certain hormones, including insulin. Pancreatic neuroendocrine tumours (PNETs) are rare tumours arising from these cells. They are classified as non-functioning (NF-PNET) or functioning (F-PNET). Functional neuroendocrine tumours cause significant symptoms due to the excessive secretion of hormones such as insulin, gastrin, or glucagon. The transformation of NF-PNETs to F-PNETs is a ...

Primary hyperparathyroidism (PHPT) is commonly the first and most frequent clinical manifestation in MEN1 occurring in up to 95% of patients. Surgery is considered the treatment of choice. Clinical course differs to that of sporadic PHPTH in terms of age of onset, complications, surgical management and recurrence rates. This study aims to review the challenges of PHPT in MEN1. Retrospective analysis of patients attending a dedicated MEN1 clinic in the Royal Victoria Hospital, ...

Introduction: Pancreatic resection for Neuroendocrine tumour is associated with a higher risk of anastomotic leak and post-operative pancreatic fistula (POPF). Aim(s): The aim of this study was to compare POPF rate after Whipple’s operation for neuroendocrine tumour (NET) versus pancreatic ductal adenocarcinoma (PDAC). Materials and Methods: We conducted a retrospective case control analysis of a prospectively maintained datab...

The Beatson West of Scotland Cancer delivers all PRRT to patients in Scotland. Approximately 4 patients are treated per week in a ward specially designed for the administration of radionuclide treatments. Pre treatment assessment is an essential part of the care and the patient in this case was identified as fit, well with bloods within normal range and a performance status of 0. Within a few hours of treatment administration the patient became unwell and was found to have col...

Introduction/Background: 16th May 2024 NICE confirmed use of Selective Internal Radiation Therapy (SIRT) for neuroendocrine tumours that have metastasised to the liver, with arrangements in place for clinical governance, consent and audit. This was subject to Commissioners/providers implementing the guidance. Trans Arterial Embolisation (TAE) and Trans Arterial Chemo Embolisation (TACE) are two of the comparative therapies that the committee examined to come...

Introduction: Incidental Coeliac artery dissection (CoAD) is an uncommon presentation of abdominal pain akin to post-prandial pain of mesenteric ischemia. Risk factors include hypertension, smoking, dyslipidaemias, pregnancy, atherosclerosis, trauma, or fibromuscular dysplasia. Imaging features of CoAD include an intimal flap, aneurysmal changes, mural thrombus, and (or) fat stranding. We report 2 novel cases of CoAD in patients with small bowel NETs with mesenteric masses.</p...

Background: Gastric neuroendocrine tumours (g-NETs) constitute about 7% of digestive NETs, with type 1 g-NETs, linked to chronic atrophic gastritis, accounting for 80% of cases. These tumours are generally small, low-grade, and exhibit excellent long-term survival rates. Increased endoscopy and awareness have raised their incidence, prompting interest in management strategies. Current guidelines recommend either endoscopic surveillance or resection for type 1 g-NETs <10 mm...

Background: Rectal neuroendocrine tumours (r-NETs) are the second most common neuroendocrine tumours in the digestive tract, with increasing incidence due to improved colonoscopy and cancer screening. These small, yellowish, low-grade lesions are often found incidentally during colonoscopies and generally have a favourable prognosis, with median survival sometimes exceeding 30 years. However, up to 20% of r-NETs may be misdiagnosed during endoscopy, risking inadequate treatmen...

Introduction: Primary and metastatic testicular neuroendocrine tumours (por mTNETs) are rare and account for less than 1% of testicular tumours. Testicular mass is the commonest presentation, with radical orchidectomy being treatment of choice. Carcinoid syndrome is rare at 1-3 % of cases. Carcinoid heart disease is even more rare in TNET but notably the right testicular vein drains into inferior vena cava (IVC) and left testicular vein drains into left renal vein then IVC byp...

Background: In southeast Scotland patients who have undergone resection of small intestinal neuroendocrine tumours (SI NETs) with curative intent are followed up by the NET team for recurrence. ENETs guidelines are that this should involve cross sectional imaging and hormonal assessment. During follow up we identified a number of patients with residual disease post surgery, rather than developing metachronous metastases. In 2019 we changed our policy to arrange post-operative ...

Background: Liver transplantation is now recognized by ENETS as a valid indication for liver-metastatic gastro-entero-pancreatic (GEP) neuroendocrine tumors (NETs). Since 2021, the UK initiated a pilot program for selected patients — specifically, primary amenable to complete resection, low-grade, well-differentiated GEP-NETs, with 6-months stability and <50%-liver-involvement.1-3Case-summary: A 34-year-old woman, ECOG-PS-0, non-smoke...

Background: Structured patient education has been used in chronic medical conditions such as diabetes mellitus, demonstrating both acceptability and improved outcomes. Here, we pilot the concept of group-based nutritional and dietary education in patients with NEN, delivered by specialist experts.Aim: To evaluate 3 outcomes: 1) acceptability, 2) effectiveness and 3) improving accessibility to specialist experts in group education settings, advising on nu...

Introduction: Mixed non-neuroendocrine and neuroendocrine neoplasms (MiNENs) of gastrointestinal tract are rare tumours with characteristic histological features include at least 30% of both neuroendocrine and non-neuroendocrine components of the whole neoplasm. They are usually treated with surgical resection and chemotherapy in advanced disease. However, there is scarcity of data for the use of peptide receptor radionuclide therapy (PRRT) in these tumours.<p class="abste...

Introduction: Neuroendocrine neoplasms (NENs) represent a diverse group of tumours with varying clinical presentations and outcomes. Despite advancements in treatment, there remains a need to better understand the behaviour of somatostatin receptors (SSTRs) to monitor disease progression and therapeutic efficacy. Our study aims to evaluate the impact of octreotide acetate (OA), Lanreotide acetate (LA) and Pasireotide (P) on LCC-18 cells, investigating potential mechanisms behi...

Introduction: Metastatic well-differentiated rectal neuroendocrine neoplasms (NENs) are heterogeneous and considered ’immune-cold.’ Standard treatments include somatostatin analogues, peptide receptor radionuclide therapy and chemotherapy. We report a case where a patient responded to third-line immunotherapy, despite the absence of predictive biomarkers.Case Presentation: A 60-year-old man was referred for colonoscopy following bowel cancer sc...

Introduction: Patients with neuroendocrine tumours (NETs) and carcinoid syndrome are at risk of developing carcinoid heart disease (CaHD). Sarcopenia is a muscle disease, associated with poorer overall survival and post operative complications in other types of cancer. In patients with NETs sarcopenia is highly prevalent, if this effects complications and survival following surgery for CaHD is unknown.The aim of this study is to describe the prevalence of sarc...

Background: Pancreatic Neuroendocrine tumours (panNETs) are usually sporadic and non-functioning, but occasionally associated with germ-line mutations causing multiple hereditary endocrinopathies. Small, non-functional lesions may be managed conservatively. For panNETs larger than 2 cm the mainstay of treatment is surgery (2). However, postoperative complications are common and can significantly impact patients’ quality of life. The aim of this audit was to explore postop...

Introduction: European Neuroendocrine Tumour Society guidelines advocate the use of platinum-based chemotherapy in combination with Etoposide as first line chemotherapy in advanced GEP-NEC. Following this regime, 30% of digestive NEC and 60% of colorectal NEC showed a lack of benefit or evidence of disease progression. Available retrospective evidence is from small studies. Other studies have shown BMI relates to survival in NEN. This study aims at studying chemotherapy regime...

Introduction: Neuroendocrine tumours can metastasize to the bones, but rarely to the orbit. The majority of orbital metastasis are incidental findings on somatostatin receptor imaging. Symptomatic metastases are treated with external beam radiotherapy or chemotherapy, seldom with surgical resection (1–4). Here we present a case who presented with visual disturbance due to an orbit metastasis which was resected and the histology showed a metastatic NET. <p class="abste...

Introduction: Until recently, lymph node metastases (LNM) in small bowel neuroendocrine tumours (SB-NETs) were classified as absent (N0) or present (N1). However, the American Joint Committee on Cancer (AJCC) updated the classification to N0, N1 (<12 positive lymph nodes), and N2 (≥12 positive LNM and/or a large mesenteric mass (MM) ≥2 cm). This updated N-classification has not been evaluated in a real-world population of patients with SB-NETs. The aim of this ...

Background: Pancreatic Enzyme Replacement Therapy (PERT) is the gold standard treatment for Pancreatic Enzyme Insufficiency (PEI), regardless of underlying aetiology. Historically, there have been intermittent PERT supply issues, however, over the past year (2023/4) this has become a more persistent and significant issue: leading to questions being raised in both UK and EU parliaments and main media reports. PERT is potentially life-saving: for those with certain conditions, i...

Merkel cell carcinoma (MCC) is a high grade cutaneous neuroendocrine tumour. They are a rare and often aggressive form of skin cancer, with increasing incidence in the elderly. MCC often presents as a firm, red/purple – coloured painless nodule with a short, aggressive history of increasing size. Wide local excision recommends 1-2cm margins, and deep clearance beyond that usually advised for keratinocytic skin cancers, but can have major cosmetic and functional implicatio...

Introduction: Many patients who present with symptoms of possible neuroendocrine tumour, start their journey with cross sectional scans to look for malignancy as part of their diagnostic workup. Often, especially with advances in functional imaging, subtle abnormalities are now visible causing diagnostic challenges. Our case details one such potential pitfall and acts as a reminder to consider broad imaging differentials when reviewing patients scans.Cas...

Background: We have seen an increasing number of carcinoid flare events following PRRT which is a recognised complication of medical/ surgical intervention. Flare events are less severe than carcinoid crisis but require prompt management to avoid patient morbidity and mortality. The rise in this complication is likely due to the complexity and advanced stage of disease at time of therapy commencement. There is very little in the literature about management of this cohort of p...

Introduction: Gastroentropancreatic neuroendocrine tumours (GEP-NETs) have been observed to manifest a variable degree of heterogeneity, resulting in varied progression, change in grade, and treatment response over time. Clinical case: A 43-year-old male presented with anorexia, weight loss, vomiting, diarrhoea, and abdominal pain. Initial imaging identified a pancreatic mass with liver and retroperitoneal nodal metastasis. The liver lesions expressed Cy...

Introduction: Small bowel NETs (SBNETs) are a group of heterogenous tumours that can exist as unifocal or multifocal disease. The current literature regarding multifocal SBNETs includes small patient cohorts and mainly focuses on histopathologic characteristics only. Data regarding the presenting symptoms and survival are lacking.Aim: To present clinical characteristic, disease free survival (DFS) and overall survival (OS) in patients with multifocal SBN...

Background: Tuberous sclerosis (TSC) is an autosomal dominant condition which can increase the risk of pancreatic neuroendocrine tumours (pNETs) (1). Patients with TSC undergo screening for renal tumours which may include pancreatic imaging (2). There are no screening guidelines for pNETs in patients with TSC.Case presentations: We reviewed four female patients who had TSC and pNET (table 1). The mean age at pNET diagnosis was 39.5 years. TSC was diagnos...

Introduction: Neurofibromatosis type 1 (NF-1) (1/3000 live births) is a rare genetic cause for hereditary pheochromocytoma (0.1-5.7%) with unclear incidence and risk of malignancy. There is no current UK screening program for patients with NF-1 for pheochromocytoma/paraganglioma.Methods: A retrospective review of patients who were diagnosed with pheochromocytoma in the context of NF-1 following referral to the adrenal multidisciplinary team at Oxford.</p...

Introduction: Malnutrition is a common problem amongst patients with gastro-enteropancreatic neuroendocrine tumours (GEP-NETs). A recent study from our group reported 75% of patients with GEP-NETs treated with monthly somatostatin analogues (SSAs) are malnourished (1). Ideally, all malnourished patients are offered nutritional support. However, guidelines and support are often lacking. The natural course of malnutrition in patients with GEP-NETs is unknown.The aim of t...

Introduction: Pancreatic neuroendocrine tumours (PanNETs) are the second most common form of pancreatic cancer. Lesions larger than 2 cm are commonly selected for surgical clearance according to the European Neuroendocrine Tumour Society (ENETS) guidelines. In this study, we assessed the variables that may affect recurrence following resection of PanNETs.Methods: All the patients who underwent resection of PanNET over a 5-year periods between 2017 –...

Objective: Neuroendocrine tumours (NETs) are rare tumours and require treatment in specialist centres with familiarity management, particularly mitigation of perioperative risk from carcinoid crisis. This study retrospectively analysed the perioperative management of NET focusing on somatostatin analogue (SSA) utilisation. It aimed to evaluate compliance with local and UKINET guidelines and assess adverse outcomes from non-compliance. UKINET guidelines tailor SSA dosing to tum...

Insulinomas are rare, functional neuroendocrine tumours (NETs) that secrete excessive insulin. They are often diagnosed late due to their variable presentation and rarity. While predominantly solitary and benign, a minority of insulinomas can present as malignant. Insulinomas typically carry a hallmark clinical presentation characterised by Whipple’s triad; symptoms of hypoglycaemia (e.g., sweating, palpitations, headaches, confusion), documented low blood glucose levels ...