UKINETS2024 22nd Annual Meeting of the UK and Ireland Neuroendocrine Tumour Society 2024 Poster Presentations (33 abstracts)
Challenges in diagnosis and treatment in an evolving pancreatic neuroendocrine tumour
Amina Adil Al-Qaysi 1 , Shani Mathara 1 , Christine May 1 , Eve Fryer 2 , Eleanor Young 2 , Mike Tadman 2 , Zahir Soonawalla 2 , Gerard Andrade 2 , Bahram Jafar-Mohammadi 1 , Aisha Naseer 2 & Aniko Rendek 2
1OCDEM, Oxford, United Kingdom. 2Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom
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 Cytokeratin and A1AT along with the DPAS positive cytoplasmic granules which favoured a diagnosis of Acinar cell carcinoma. However, a strong expression of both Synaptophysin and Chromogranin was also seen with Mib-1 proliferation index of 10%, and this was classified as an Acinar cell carcinoma. The disease progressed despite chemotherapy directed at this pathology. The patient started to experience recurrent hyper-insulinemic hypoglycaemic episodes two and half years after presentation. A repeat liver biopsy revealed a well-differentiated Grade 2 Neuroendocrine Tumour (NET) with Ki-67 11% and focal positivity for Insulin, suggestive of metastatic insulinoma (initial biopsy negative immunohistochemistry for Insulin in retrospect). All disease sites were somatostatin receptor avid on Ga68 DOTATATE PET-CT scan. The hypoglycaemic episodes proved challenging to treat, despite, Diazoxide, steroids, and somatostatin analogue (SSA) therapy. He was treated with four cycles of Peptide Receptor Radionuclide Therapy (177Lu-DOTATATE), with good symptomatic response which enabled an uncomplicated right hepatectomy. He continues SSA therapy and is planned to have distal pancreatectomy and splenectomy.
Conclusion: Heterogeneity and variable GEP-NETs progression has been reported, though is not fully understood yet. Our case highlights the diagnostic challenges and the change in functionality and behaviour of pancreatic NETs. Our case also demonstrates the multimodal therapies and multidisciplinary teams that are needed in the treatment of such challenging cases. Future research is needed to identify the predictors of this biological behaviour, aiming to provide personalised NET therapy.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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