Dr John Bevan
University of Aberdeen

This workshop was well attended by patients and family members, reflecting the great interest in, and need for support, for people with this unusual and difficult condition. The workshop provided a clear and detailed overview of craniopharyngiomas, which stimulated a lively discussion of the challenges faced by patients and families.

Craniopharyngiomas are rare compared with most medical conditions, with only one to two new cases per million of the population per year. Although about half of new cases occur in children, craniopharyngiomas can come to light for the first time in adults. They are rare benign tumours derived from remnants of Rathke's pouch and are made of solid tissue, cysts and calcified nodules. Symptoms occur due to a combination of direct pressure effects on the brain and disruption of the normal production of hormones from the Pituitary gland. The first of these commonly causes visual loss as a result of pressure on the optic nerves, which connect the eyes to the brain, and disturbs an important brain area known as the Hypothalamus. Damage to this brain region is associated with hydrocephalus - a build up of fluid that has to be relieved by an operation to insert a special valve. When the hypothalamus is damaged the normal control of appetite and body weight are often affected, sometimes leading to uncontrollable appetite - so-called hyperphagia.

Dr Bevan described the various investigations and showed a series of magnetic resonance (MR) brain scan images of patients with craniopharyngiomas, before and after surgery. He also gave a vivid illustration of a water deprivation test used in the diagnosis of Diabetes Insipidus - a common problem for people with Craniopharyngioma. Many treatment challenges were highlighted, including the young age of many patients, the complicated and sometimes prolonged treatment involving surgery (sometimes several operations), Radiotherapy and hormone replacement, fertility issues, and long-term worries about body weight and self-image.

The discussion that followed illustrated the wide age range of those affected, their many concerns, the long-term and difficult nature of the problems that can result, and the wide spectrum of their severity. This spectrum ranges from degrees of hypopituitarism, which is usually well controlled with replacement of pituitary hormones, through to more severe damage to the hypothalamus and surrounding brain areas, associated with weight gain, and sometimes complex behavioural changes. The issue that generated most questions was that of weight gain. The majority of affected people attending this workshop had experienced weight gain from the time of diagnosis and treatment. While this was sometimes relatively minor, it had been a major problem for many. For some, weight gain was associated with frank hyperphagia, while others had experienced weight gain without apparently changing their eating habits in any way. Interstingly, several participants commented that they had experienced marked cravings for sweet food. The merits of different diets were discussed, although there has been no research on this issue. The importance of pituitary hormone replacement for full energy and controlling weight gain was emphasised, including the importance of adequate doses of Thyroxine and, when indicated, Growth hormone. Several participants drew attention to their experiences of weight loss with craniopharyngioma, as if it were mimicking anorexia nervosa. Although weight gain is the more common problem in craniopharyngioma, weight loss is also a recognised consequence of hypothalamic damage.

The workshop highlighted the main areas of unmet need, particularly for better information, and greater personal and family support for coping with long-term problems caused by the condition.