Clinical Use: Diagnosis and subsequent monitoring of a diverse group of neoplasms that arise from the endocrine and nervous systems. The large number of potential serum tumour markers associated with tumours of this type necessitates a panel of markers being assembled resulting in the screens listed below. Patient symptoms may aid the clinician in selecting the appropriate screen.
Background:

Neuroendocrine Tumours – NETs are a group of tumors that can be a challenge to diagnose. Common features are similar morphology, presence of secretory granules and the production of biogenic amines and polypeptide hormones. Prevalence is 35/100,000 of which two-thirds are carcinoid tumours. Certain NETs can produce vague, nonspecific symptoms (such as diarrhea, abdominal pain, flushing, and hypoglycemia) that are easily mistaken for other conditions, such as irritable bowel syndrome and inflammatory bowel disease.NET 2 can form in multiple locations throughout the body, including difficult-to-pinpoint areas within the mid gut. It is not unusual for patients to suffer for years before a NET is correctly diagnosed. The majority of NETs originate in the gastrointestinal tract or pancreas, and are collectively referred to as gastroenteropancreatic NETs (GEP-NETs). There are several different types of GEP tumours (gastroenteropancreatic tumours). They tend to develop in the organs of the digestive system such as the stomach, intestines and the pancreas. NETs that are not gastrointestinal or pancreatic in origin can arise in the respiratory tract, ovaries, and testes, as well as in other endocrine tissue (e.g. thymus, adrenal gland). Also included in the NET classification are some inherited conditions including Multiple Endocrine Neoplasia type 1 (MEN1), MEN2, Neurofibromatosis and Von-Hippel-Lindau (VHL) disease.

Multiple Endocrine Neoplasia is a rare genetic condition that is caused by a faulty gene and is usually hereditary, although not always. There are two types – MEN1 and MEN2, they are completely separate conditions with different genetic causes. MEN1 tumours can be non-cancerous (benign) or cancerous (malignant). Almost all people with MEN1 will develop hyperparathyroidism at some stage in their life. There may be tumours in the parathyroid glands, which are usually benign. Tumours can also develop in the digestive system (gastrointestinal tract or GI tract), usually in the stomach, intestines, pituitary and/or adrenal glands and/or pancreas. Rarely, people may develop a particular type of tumour (known as a carcinoid tumour) in the chest, thymus gland (behind the breast bone), or the gastrointestinal tract.

Many people with MEN2 develop medullary cell carcinoma of the thyroid. This is a malignant tumour. Some people who have MEN2 will develop Pheochromocytoma. Patients with MEN 2A commonly develop hyperplasia of the parathyroid glands. People with MEN 2B develop benign neuromas that appear as swellings on their tongue, lips, eyelids, or lining of their mouths. In familial MTC, people tend to get medullary thyroid cancer only.

Reference Ranges: 0 – 150 ng/L
Associated Diseases:
Patient Preparation: For suspected gastrinomas, patient off proton pump inhibitors for 2 to 3 days, then request Gastrin, Chromogranin A and Pancreatic Polypeptide.
Specimen Requirements: Please see either Neuroendocrine Tumour (NET2) Screen, Multiple Endocrine Neoplasia Screen, Carcinoid Screen or Endocrine Diarrhoea Screen for relevant specimen requirements
Turnaround Time: 2 weeks
Time Limit for Retrospective Requesting: N/A
Additional Information: www.labtestsonline.org.uk
Referred Test: Referred Test
Location: Clinical Biochemistry Department
Royal Victoria Infirmary
Queen Victoria Road
Newcastle-upon-TyneNE1 4LP