Acromegaly, or Pierre Marie’s disease as it is sometimes called (after the first doctor to accurately describe the symptoms), is a hormonal disorder characterized by abnormal bone growth, enlarged hands and feet, and thickened, enlarged face. Learn more about this rare endocrine disease, its diagnosis and the different treatment options available.
Treatments focus on two objectives:
- Removing the tumor and alleviating the symptoms,
- normalizing growth hormone production and halting the progress of the disease.
There are two types of treatment available:
Surgery is the most common option to remove the pituitary tumor. The operation is often carried out using an incision through the nose, but it may sometimes be necessary to open up the skull. The surgery returns growth hormone production to normal in 90% of cases with a small adenoma, and in 50 to 60% of cases for adenomas with a diameter of more than 10 mm.
When surgery is not an option, or if surgery does not successfully normalize the levels of IGF1 or growth hormone, of if the patient relapses, other potential treatments include radiotherapy or medication.
When surgery is not successful, drug therapies are used to lower production of growth hormone and/or IGF1 by the tumor. The drug families in the treatment of acromegaly are:
- somatostatin analogs, especially octreotide and lanreotide, to normalize secretion of growth hormone and shrink the tumor size from the first three months of treatment. The main side effects of these drugs are gastrointestinal problems, such as abdominal pain, diarrhea, or the presence of excess fat in the feces (steatorrhea).
- Pasireotide is a somatostatin analog that has recently been approved in the treatment of acromegaly. The main side effect of pasireotide is that it can cause hyperglycemia and sometimes even cause diabetes mellitus.
- Dopamine agonists, like bromoocriptine and quinagolide, reduce the secretion of growth hormone by the tumor. One of the potential side effects of this drug family is digestive disorders, including nausea and vomiting, drops in blood pressure when moving from lying down to standing – which can lead to fainting – and pronounced headaches.
- Pegvisomant acts to block the effect of GH on its receptor. It can be used alone or in combination with somatostatin analogs when these are not sufficiently effective in controlling the levels of growth hormone or IgF1. It is administered daily by subcutaneous injection.
Patients may consult with a number of different specialists, depending on the symptoms and the progress of the disease: cardiologist, diabetologist, ophthalmologist, and a rheumatologist may also be called in.
3 to 4
New cases of acromegaly per year per million population.
Average age of diagnosis
Fédération d’endocrinologie du Pôle Est. Website: http://federation-endocrino-poleest.univ-lyon1.fr/webapp/website/website.html?id=3300926&pageId=220550
Philippe Chanson. Acromégalie. Presse Med. 2009, 38: 92-102. http://www.em-consulte.com/showarticlefile/197873/main.pdf