GRFoma
ORPHA:97261· ICD-10 E16.8
Definition
GRFoma is a type of pancreatic endocrine tumor that hypersecretes growth hormone-releasing factor (GRF or GHRH) and that clinically resembles a pituitary adenoma as patients present with acromegaly. In addition to the pancreas, this tumor can also occur in the lungs or small intestine, are usually large > 6cm and approximately 1/3 have metastasized at the time of diagnosis. It often co-occurs with Zollinger-Ellison syndrome or multiple endocrine neoplasia type 1 (MEN 1).
- Prevalence
- Unknown
- Inheritance
- Not applicable
- Age of onset
- Adult