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Tumor causing gigantism under control long term

People with an extreme form of acromegaly, commonly known as gigantism, benefit from combination therapy, also in the long term.

Acromegalie - Rigardus RijnhoutErasmus MC, which treats a majority of the difficult to treat acromegaly patients in the Netherlands, has been using this treatment for ten years. But now PhD student Sanne Franck has discovered that the combination therapy continues to work well even after 10 years. She will earn her PhD on 15 March.

Acromegaly is a rare disease in which a tumor grows on the pituitary gland. The pituitary gland  is found in the brain and  produces and helps regulate hormones in the body. One of the hormonal processes that it regulates is growth. The pituitary tumor in people affected by acromegaly produces excess growth hormone, which means that patients can become abnormally tall if the disease occurs before puberty.

Other features include abnormally large hands and feet and  large, prominent facial features. The tumor is often discovered late, which means that complete surgical removal  of the tumor may no longer be possible.

It is estimated that about 1,600 people in the Netherlands are affected by acromegaly. Each year, there are approximately 160 new patients. Less than half of these patients can be cured by surgery, whereby the tumor is removed through the nose. For the majority of patients this is not possible because the tumor is already too large. Remnants of the tumor will remain and these will need to be treated using drugs to control the hormone production by the tumor.

In about 40 percent of the patients the tumor can be controlled by somatostatin analogues, a hormone-like substance that suppresses the hormone production by the tumor. The vast majority of the patients (97%) for who this single-drug therapy does not work well enough, therefore benefits from combined treatment with somatostatin analogues and pegvisomant, a growth hormone receptor antagonist.

Erasmus MC is highly specialized in the treatment of acromegaly. Erasmus MC's department of Endocrinology has the largest group of patients worldwide being treated with the combination therapy, which makes it a highly suitable location to study the effect of the treatment. "Ten years ago, we started the combination treatment with pegvisomant and somatostatin analogues. But you always have to wait and see whether a treatment remains effective over time. This appears to be the case," says Sanne Franck.  

Although acromegaly is a rare condition, there are a number of patients who became world famous thanks to the disease. Take, for example, the American actor Richard Kiel who passed away in 2014 and is known for his role as 'Jaws' in the James Bond films 'The Spy who loved me' and 'Moonraker'. In the 1940s and 1950s Rigardus Rijnhout enjoyed international fame as the "Giant of Rotterdam'.

Date published: 13 March 2017.

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