Bringing Excellence to Life

Familial endocrine neoplasia syndromes

The familial endocrine neoplasia syndromes that we treat in children include:

Multiple endocrine neoplasia (MEN) 

Endocrine neoplasia syndromes refer to a group of disorders in which tumours (almost always benign) develop in various hormonal glands, depending on the form of the condition. The three forms are multiple endocrine neoplasia (MEN) I, MEN IIa and MEN IIb; each of these is genetically inherited. All are complicated conditions requiring very detailed and expert evaluation. Detecting and treating these tumours is extremely important and our aim is to treat these tumours as early as possible. We have close links with experts in genetics from Great Ormond Street Hospital for children who, in many cases, are able to test directly for the change in DNA that is responsible for the condition. 

Treatment options are often complicated and decided upon within an expert multidisciplinary team. Surgical removal of tumours is possible but for many patients long term tablet treatment is also suitable.

For more information on MEN, or to contact a national support group, please click here

Von Hippel-Lindau syndrome (VHL)

Von Hippel-Lindau syndrome is an inherited condition in which a variety of tumours develop around the body. In people diagnosed with VHL, their blood vessels grow into little knots (normally they would grow like trees).  These growth knots are called angiomas or hemangioblastomas and they may develop in the eye retina, certain areas of the brain, the spinal cord, the adrenal glands or other parts of the body.  Detecting and treating VHL early is important and, as with MEN, our aim is to treat the knot-like growths while they are small and before they do permanent damage.