Parathyroid hormone‐related peptide in pancreatic neuroendocrine tumours associated with hypercalcaemia

• Published in: HPB (Oxford, England) England), 2001-09, Vol.3 (3), p.221-225
• Main Authors: Papazachariou, I.M., Virlos, I.T., Williamson, R.C.N.
• Format: Article
• Language: English
• Subjects: Hypercalcaemia; Pancreatic Neuroendocrine Tumour; Parathormone; Parathyroid Hormone-RELATED Peptide
• ISSN: 1365-182X; 1477-2574
• DOI: 10.1080/136518201753242253

Hypercalcaemia is a common paraneoplastic syndrome. In the context of pancreatic neuroendocrine tumours, it is occasionally caused by secretion of parathyroid hormonerelated peptide (PTH‐rP). Two patients are reported in whom persistent hypercalcaemia was traced to a large neuroendocrine pancreatic tumour hypersecreting PTH‐rP. Resection of the tumour reduced serum levels of calcium and PTH‐rP transiently in each case until the patient developed bulky metastatic disease. A 33‐year‐old woman remained hypercalcaemic after the removal of all four hyperplastic parathyroid glands had rendered circulating parathormone levels undetectable. Radical distal pancreatectomy was followed over the next 4 years by operative debulking of liver metastases, multiple hepatic artery embolisations, octreotide injections and repeated admissions for intravenous fluid and biphosphonate therapy. A 41‐year‐old man presented with hypercalcaemia as well as features of somatostatinoma syndrome. Symptomatic improvement after radical distal pancreatectomy was short‐lived, and hepatic artery embolisation failed to control his rapidly progressive disease. Malignant hypercalcaemia associated with a neuroendocrine pancreatic tumour hypersecreting PTH‐rP is difficult to treat and can be life‐threatening. Aggressive surgical treatment is recommended initially, while somatostatin analogues and hepatic artery embolisation are alternative therapeutic options for metastatic disease.