Association for
Multiple Endocrine
Neoplasia Disorders

Pituitary Tumours

The pituitary gland can be found at the base of the brain behind the nose, in a bony box called the sella turcica . It is very close to the optic nerves from the eyes and therefore, occasionally pituitary tumours (also called adenomas) can cause problems with vision. They may also cause headaches. 3 out of 10 (30%) of MEN1 patients will develop a pituitary tumour.

There are several different types of benign (non-cancerous) pituitary tumour, which can differ in size (microadenomas of less than 1cm in diameter, or macroadenomas of more than 1cm). All forms of pituitary tumour may be detected using an MRI or CT scan of the head as well as blood tests. Treatment may be in the form of medication or surgery. This will depend upon the type of tumour and its size. Sometimes small tumours can be treated with tablets or injections although often surgery is needed.

The operation to remove a pituitary tumour is a relatively small one, and although carried out under general anaesthetic it does not involve having to operate on the main part of the head. Pituitary growths are not brain tumours.

Parathyroid Tumours

Parathyroid glands are small glands lie just next to or are sometimes embedded within the butterfly-shaped thyroid gland in the neck. More than 9 out of 10 (90%) of MEN1 patients will develop a parathyroid growth. These growths release large amounts of parathyroid hormone (PTH) and so can be easily found using blood and urine tests.

Some symptoms of an untreated parathyroid growth include irritability, ulcers, kidney stones, pancreatitis, bone fractures, osteoporosis, tiredness, muscle or bone pain, indigestion, poor memory and constipation. However most patients will have very few of these symptoms particularly when diagnosed and treated early on.

Treatment involves removing the glands with tumours by surgery. These days, most surgeons will remove most of the parathyoid glands and just leave half of one gland (remnant) behind to help control the body’s calcium levels (sub-total parathyroidectomy).

After parathyroid glands are removed, patients may have to take a Vitamin D capsule to help the body absorb calcium.

Duodenopancreatic Neuroendocrine Tumours

The pancreas lies in the abdomen behind the stomach and is connected to a part of the small intestines called the duodenum. It produces several hormones important for the body’s energy levels. 3 out of 4 (75%) of MEN1 patients will develop one of several pancreatic neuroendocrine tumours and 1 in 10 (10%) of those will develop more than one type of tumour. While most of the tumours will produce large amounts of hormones, some may not be active (non-functioning). Up to half of the tumours may become malignant (cancerous) if left untreated. Most pancreatic tumours are found using an MRI scan and blood tests. If the tumours are very small, other types of scan may be used.

Treatment depends on the size and type of tumour. Anti-ulcer medications can be used to control symptoms of tumours called Gastrinomas, but other types of tumour are usually treated with surgery. The operation may remove just the tumour, or remove the whole or a part of the pancreas. If the whole or a major part of the pancreas is removed, a patient will then have diabetes, which must be treated with regular insulin injections. Please note that the neuroendocrine pancreatic tumours of MEN1 are NOT the same as the more common pancreatic cancer, adenocarcinoma.