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Pancreatic neuroendocrine tumours
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== '''Specific situations''' == * Patient presents with symptoms ** Functional testing and localise the tumour ** Surgical resection ** Approach and extent dictated by tumour type and patient factors ** Mostly perform partial pancreatic resection with lymph node sampling * Patient presents with lesions found incidentally ** Work up with imaging - local and systemic staging with CT CAP, DOTATATE PET, consider EUS +/- FNA ** If <2cm and G1, observation may be an option - repeat CT/MRI at 6-12 months with surgery if increase in size by 0.5cm or more ** If >2cm or G2 or G3, generally recommend resection in a good operative candidate * Liver mets ** Liver resection - either curative or palliative ** Resection recommended if >90% of disease can be removed ** 80% recurrence at 5 years, but 85% 5-year survival ** Factors with better prognosis: *** G1 or G2 tumours *** Absence of distant lymph node metastases *** Absence of extrahepatic or peritoneal metastases ** Palliative: can do HAE/chemoembolisation/RFA, but outcomes appear to be worse, not curative ** Liver transplant has been proposed under certain circumstances (unresectable neuroendocrine metastases) but this is controversial, and not done in Australia ** Debulking is reasonable if >90% of tumour can be removed * Metastatic PNETs in general ** Somatostatin analogues - effective in controlling both hormone secretion and stabilising tumour growth *** Can provoke cholelithiasis - if possible, cholecystectomy at initial operation ** Knowing the type of somatostatin receptor expressed by the tumour can help guide therapy - there are 5 different types ** Cytotoxic chemotherapy is also an option, especially for poorly-differentiated PNETs
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