Pancreatic neuroendocrine tumours (or PNETs) are rare neuroendocrine lesions that can develop within the pancreas. They are unique in that they may secrete excessive hormones depending on the specific type of tumour and can be one manifestation of genetic syndromes (eg. multiple endocrine neoplasia, Von Hippel Lindau or neurofibromatosis).
The vast majority of PNETs however are what are called non-functional and are typically slow to grow. They key is early identification, but unless you are aware of a familial or personal risk factor for the disease this is difficult to achieve. If presenting with symptoms (eg. pain or jaundice), this usually means that the lesion has grown to a size where local invasion into surrounding structures may have already occurred. Dedicated blood tests and imaging investigations can assist defining the nature and extent of the disease and is often crucial when planning treatment which will often involve surgery. Conversely, if identified early and defined as a small, low grade tumour, often ongoing imaging surveillance over time is all that is necessary.
Functional tumours typically present owing to excess hormone secretion and the specific type of functional PNET is typically named according the specific hormone in question. Insulinoma (insulin excess) and gastrinoma (gastrin excess) are typical examples. The treatment of such lesions generally involves surgical excision to cure the problem and eliminate the hormone excess.
In general, PNETs are generally slow growing tumours and patients enjoy a good outcome. As with all pancreatic problems, it is key that you are attended to by specialists who not only understand what to do, but why, based on their in depth understanding of the disease itself.
