Pituitary News, Issue 9 - Autumn 1998.

Craniopharyngiomas are rather uncommon benign tumours, which account for between 4% and 9% of brain tumours in childhood and 1% in adults. Craniopharyngiomas can cause symptoms at any age, from the newborn period to old age, depending on the tumour's rate of growth, but in about 50% of cases, the first effects are noticed in childhood, most commonly between the ages of 5 and 12.

Often patients have had symptoms for many months when they first go to a doctor. Symptoms are usually related to the pressure effects of the slowly-growing tumour, such as headaches and visual problems. Endocrine disturbances can arise from pressure on the pituitary, stalk or Hypothalamus, and can cause a number of problems, including impaired growth in children and also Diabetes Insipidus. The hypothalamus, in addition to relaying messages from the brain to the pituitary, has many other functions, including controlling appetite, so damage to it may lead to difficulty controlling food intake, resulting in obesity. Short-term memory may also be affected, and some patients experience mood swings and sleep disturbance.

Once the condition is suspected, diagnosis is usually relatively straightforward. Before CT and MRI scans were available, the diagnosis was often made from a plain skull X-ray, as the calcium in the tumour made it easily visible. Nowadays, MRI scans are the best way of 'seeing' the tumour. Blood tests will also be carried out to check the function of the pituitary, and visual tests will usually be carried out. Treatment is usually provided jointly by an Endocrinologist and a neurosurgeon, but an ophthalmologist, psychologist and other specialists may also be needed.

Treatment is usually surgical, carried out through a craniotomoy (lifting of a skull flap), aiming to remove all or part of the tumour. In some cases, it is not possible to remove the whole tumour and, in these patients, partial removal followed by Radiotherapy (to prevent regrowth) is usually effective. In some patients it may be more appropriate to first relieve any pressure build-up caused by the tumour by inserting a shunt or, if the tumour is mostly cystic, it may help to aspirate fluid from the tumour.

Unfortunately, tumours sometimes grow back after surgery, so patients need long-term follow-up by an endocrinologist. Children will normally be looked after by a specialist paediatric endocrinologist and later transferred to an adult endocrinologist. If pituitary function has been damaged, then ongoing treatment may include replacement of one or more of the hormones produced by the pituitary. Further fluid drainage may be needed from time to time.

This is a very brief introduction to a complex topic. For more information on craniopharyngioma in children, read Serono Laboratories's leaflet, Craniopharyngioma: A Guide for Parents and Patients, produced under the auspices of the Child Growth Foundation (CGF) and the British Society for Paediatric Endocrinology and Diabetes (BSPED). This leaflet is available from the Child Growth Foundation.