Endocrine tumours of the pancreas comprise tumours which arise from the pancreatic islet cells and include a number of distinct tumours which match the cell type of origin. Each has different clinical presentation, on account of them elaborating various hormones and different biological behaviours.
These tumours can be divided according to whether or not they secrete active compounds. They are:
- functional tumours: 85%
- non-functional tumours: 15%
Individual functional tumours are discussed in more details separately. Below is a general discussion focusing on non-functioning tumours.
Tumours can be isolated or associated with the multiple endocrine neoplasia type I (MEN I), which is characterized by the triad of parathyroid, pituitary, and pancreatic lesions.
Functional tumours tend to present earlier, with clinical signs and symptoms related to their cell type.
- insulinoma: Whipple's triad
- gastrinoma: Zollinger-Ellison syndrome
- glucagonoma: glucagonoma syndrome
- non-functioning tumours: tend to present late, similar to pancreatic adenocarcinomas
Multiphase contrast enhanced thin slice cross-sectional imaging is ideal. Tumours tend to be smaller and hypervascular and may be calcified, and may be cystic.
Treatment and prognosis
If diagnosed early enough then complete surgical resection can be curable. Biological behaviour also depends on cell of origin:
- insulinoma: 10% malignant
- gastrinoma: 60% malignant
- glucagonoma: 80% malignant
- VIPoma: 75% malignant
- somatostatinoma: 75% malignant
- non-functional: 85-100% malignant
- 1. Demos TC, Posniak HV, Harmath C et-al. Cystic lesions of the pancreas. AJR Am J Roentgenol. 2002;179 (6): 1375-88. AJR Am J Roentgenol (full text) - Pubmed citation
- 2. Raman SP, Hruban RH, Cameron JL et-al. Pancreatic imaging mimics: part 2, pancreatic neuroendocrine tumors and their mimics. AJR Am J Roentgenol. 2012;199 (2): 309-18. doi:10.2214/AJR.12.8627 - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Pancreatic islet cell tumours||✓|
|Pancreatic endocrine neoplasms||✓|
|Neuro-endocrine tumours of the pancreas||✗|
|Neuroendocrine tumours of the pancreas||✗|
|Pancreatic neuroendocrine tumours||✓|
|Endocrine tumours of the pancreas||✗|