Professor Stafford Lightman, Bristol Royal Infirmary and Sue Thorn, Executive Director, Society for Endocrinology

Some time ago, Sue Thorn, together with two other pituitary patients, attended a workshop which had been organised by the Royal College of Physicians with the intention of trying to obtain agreed UK guidelines for managing pituitary illnesses (including Craniopharyngioma).

The workshop brought together many of the country's top endocrinologists, as well as pituitary surgeons, ophthalmologists, radiotherapists and GPs. A report has now been produced to summarise this work, and it has some interesting information on how the main disorders should be handled.

In addition to its general interest value, some parts of the report can be seen as providing minimum standards that you should be entitled to, and can be used to try to check that the hospital treating you has enough expertise in all the relevant areas.

In this issue of Pituitary News, we will look at some of the general parts of the report, and also at the section on non-functioning tumours. These are one of the most common types of tumour and the name means that they do not cause production of any excess hormones. In future issues, we will look at what the guidelines say about hypopituitarism, craniopharyngiomas, Cushing's, Acromegaly and Hyperprolactinaemia (Prolactinoma).

The booklet starts by outlining the various factors which have led to inconsistent patterns of patient management across the country. First, pituitary tumours are relatively rare, estimated in the report at about 20-30 new cases per year per million of population. Then there is the fact that the initial diagnosis may be made by a number of different groups, including GPs, ophthalmologists and gynaecologists, to name only three. There is apparently evidence of some repetition of tests which together with repeated visits to various specialist clinics can make it difficult to have accurate information about treatment over a period of time, as well as leading to a waste of precious NHS resources.

Before going on to consider specific disorders, the report has a list of general recommendations for the level of provision of various services to pituitary patients. The box below contains some main points which should be of immense use to patients and carers in discussing your treatment with your GP or other medical advisors.

The report also contains an admirable list of the objectives of treatment, the very first of which is that you should feel well! The full list is reproduced below, and could be a useful discussion point with your GP or endocrinologist. However, please remember that endocrinologists are not magicians. These are objectives and can be achieved in most cases, but there will always be a few, particularly complex, cases where full 'restoration of feeling of well-being' for instance, may not be possible, at least in the short-term. After all, another of the features of pituitary disease is the wide variety of symptoms and responses to treatment.

Another very welcome part of the report is the paragraph on counselling, which states that 'patients should receive thorough counselling'. It also reminds healthcare professionals of the need for 'a balance between optimism and realism'.

The general section concludes with recommendations on imaging and diagnostic tests. It states that, under normal circumstances, magnetic resonance imaging (MRI) scanning is preferable to computer tomography (CT) scanning. The group recommends that pituitary function testing is carried out only in a 'specialised investigation unit' which is well geared up for collecting samples, and preparing, analysing and interpreting results. Joint review of each patient's progress by an endocrinologist and a pituitary surgeon should be carried out until at least a year after the operation.

Non-functioning tumours

As mentioned above, these are one of the most common types of pituitary tumour and the main symptoms are often headaches, visual problems, fertility problems and generally feeling run down. About a quarter of all pituitary tumours are non-functioning. It is always worth repeating that these tumours are almost always benign. Pituitary tumours are not linked with cancer.

The report recommends a series of tests before any operation. These include formal testing of vision, and MRI scans to determine the size of the tumour and where it may have spread to. It is 'essential' that Prolactin is measured in all patients before any operation is carried out. If prolactin is high, an endocrinologist should be consulted.

Hormone tests are recommended before operation for three reasons. The first is to give any replacement which may be needed until the operation; the second is to identify the 'baseline' values without which it is not possible to measure the effect of the treatment; and the third is to ensure that it really is a Non-functioning tumour (ie that there are no excess hormones being produced).

If there are no symptoms of pressure (eg visual field abnormalities), the options are to monitor via regular MRI scans, or to operate. Radiotherapy may also be needed.

If there are pressure symptoms, urgent Transsphenoidal surgery is needed. Occasionally, a craniotomy is required (this is the larger operation where the surgeon gets at the tumour through the skull rather than through the nose or nasal sinuses). Some hormone replacement may be needed around the time of the operation. Radiotherapy may be needed afterwards.

There are some particularly encouraging facts in the section on 'outcomes and follow-up'. For instance, for many patients the visual problems will improve shortly after the operation, and headaches are relieved in over 90% of cases. Up to one fifth of patients will get Diabetes Insipidus (DI) immediately after the operation, but only a few have this permanently. Other than DI, less than one in ten patients have any complications arising from the operation.

Looking ahead, the group recommends regular eyesight assessment. They also recommend a scan 2-3 months after the operation to check whether the tumour was fully removed. Further scans to monitor this are needed after 6-12 months and then at intervals of 1-5 years. Hormone levels also need to be monitored and corrected if necessary.

As regards radiotherapy, some centres do this routinely; some only if not all the tumour could be removed; and some only if there is evidence of regrowth. As we read in Dr Plowman's article recently, the effects of radiotherapy are gradual. The report says that the effects are most pronounced in the first two years and continue slowly thereafter. By 20 years after radiotherapy, almost half of patients will have some degree of loss of one or more pituitary hormones (hypopituitarism). This is probably why many centres only give radiotherapy if they think there is a risk of the tumour regrowing. Because of this risk of hypopituitarism, if you have had radiotherapy, you will need hormonal testing at regular intervals. The report recommends 6 months after radiotherapy, after one year, after two years and then every two or three years.