Pancreatic Neuroendocrine Tumours
These are a special type of tumour—which arises within the pancreas – these arise in special type of cells – located and found within the pancreas.
The neuroendocrine cells are like nerve cells and are like hormone making endocrine cells.
Neuroendocrine tumours arise within these cells.
These tumours are not typical of pancreatic cancer (adenocarcinoma) and are biologically different.
Depending on their biology and aggressiveness pancreatic neuroendocrine tumours are classified into 3 grades
Grade 1
Grade 2
Grade 3
Grade 3 tumours are also called Neuroendocrine Carcinomas (NEC) and are known to be locally aggressive and metastasise as well .
However they do carry a potential to metastasise to different parts of the body –
Functioning versus Non-Functioning Tumours
about 10% of all pancreatic neuroendocrine tumours are functioning – they secrete hormones and this leads to specific hormone related syndromes.
- Insulinoma – in case of release of excessive insulin – which causes low blood sugar and hypoglycaemia
- Gastrinoma – these cause release of excessive amounts of gastrin and this in turn excessive and abnormal acidity – and leads to development of ulcers within the stomach, small intestine and even the gullet – food pipe.
- Some other rarer hormone secreting tumours
Diagnosis
- CT scan and imaging – the tumours have a characteristic appearance which raise the suspicion of Pancreatic Neuroendocrine tumours.
- DOTA scan : this is a specialised type of nuclear medicine scan, specific and sensitive for NET
- Presence of pick up of the dye confirms presence of tumour
Treatment
- Small asymptomatic non functioning NETs may be kept under observation and surveillance
- Surgery remains the mainstay of treatment of NETS – removal of the tumour through a standard operation is curative. The operation may involve either performing a whipple pancreaticoduodenectomy or a distal pancreatectomy (removal of the body and tail of the pancreas).
- Pancreatic NETs may also spread to other parts of the body – eg: Liver
Where possible all attempts to remove the part of the liver bearing the tumour should be done. This is potentially curative and achieves very good long term results.