Pancreatic neuroendocrine tumours are rare with an incidence of 5 per million. Of these tumours, 75% maybe hormonally functioning. They carry a better prognosis than adenocarcinoma of the pancreas. Parathyroid Hormone related peptide hypersecretion in PNETs is uncommon and is solely associated with metastatic PNETs. 31 cases are reported of PTH-rPoma in the literature. Here we describe a novel case presenting with cough. A previously well 56-year-old female presented to the acute medical take with cough and shortness of breath. Initially having been managed as asthma she was investigated for a possible acute pulmonary embolus. She was noted to be hypercalcaemic: with an adjusted calcium level of 2.68 mmol/L (NR 2.6 mmol/l). Investigations revealed the parathyroid hormone (PTH) appropriately suppressed to 1.0 pmol/l and undetectable vitamin D levels. PTH-rP levels were raised 2.0 pmol/l (NR <0.5 pmol/l). Her CTPA was negative for pulmonary embolus but revealed lesions in the liver and spleen. Further dedicated CT imaging confirmed a mass lesion in the tail of the pancreas with metastases in the liver and spleen. Tissue diagnosis was made from an USS guided biopsy of one of the liver lesions, revealing a neuroendocrine tumour of the pancreas, Ki67-7% ENETS grade 2. Octreoscan with SPECT CT demonstrated the disease was restricted to below the diaphragm: the liver /pancreas and spleen. Low grade uptake was also noted in the peritoneum. After careful discussion in the NET MDT surgery was felt to be high risk at this stage and she was commenced on Sandostatin LAR 30mg. Due to increasing somnolence Sandostatin LAR was switched to Lanreotide autogel 120 mg every 28 days which abated some symptoms. This helped to keep her adjusted calcium levels stable for 12 months. Subsequently her calcium levels were noted to have increased slightly and surveillance CT imaging showed progressive disease and assessment for Peptide receptor radionuclide therapy (PRRT) planned from the NET MDT. Predictably efficacious for both hypercalcaemia and PNET progression. In the interim there has been a dose escalation of the Lanreotide 120 mg to every 21 days. PTHrP related uncontrolled hypercalcemia is the key clinical effect of the functioning pNET and is associated to increased morbidity and mortality. There is great evidence for the targeted therapies (mTORi(everolimus) and TKI (sunitinib)) and PRRT. This case highlights the use of somatostatin analogues in managing hypercalcemia and progression in metastatic PNET and use of PRRT.
18 - 21 May 2019
European Society of Endocrinology