gms | German Medical Science

In pituitary dependent Cushing’s disease, the appropriate treatment is surgery. Despite high remission rates in the hands of a skilled neurosurgeon, recurrence of the disease is unpreventable (15%). In this case, normalisation of the elevated serum cortisol has to be achieved as soon as possible. The aim of this retrospective study is to demonstrate the options of management from the neurosurgical point of view.

A post-operative serum cortisol level below 2μg/dl after 2mg dexamethasone overnight and basal serum cortisol and plasma-ACTH levels within the normal range define the cure. Recurrence is defined as reoccurrence of clinical and endocrinological signs of the disease after complete clinical and biochemical remission. Preoperative diagnosis consists of basal serum cortisol and plasma-ACTH levels and of 2mg Dexamthasone overnight test. Sinus petrosal sampling with determination of ACTH provides no further information due to post-operative changes in venous drainage. A total of 16 patients were re-operated on recurrent Cushing’s disease from 1997 to 2002. Re-operation as the first choice of treatment represents the attitude of the authors. In the event that there was no signs of a successful re-operation, radiotherapy and/ or adrenalectomy was performed.

In 2 patients no tumour was found. One of these patients was normalised later by adrenalectomy, the other patient underwent no further treatment. Four of the remaining patients were normalised following surgery, in 10 cases Cushing’s disease was persistent following operative treatment; 3 of these patients were normalised following bilateral adrenalectomy, one was normalised following adrenalectomy and radiotherapy and 4 patients were normalised following radiotherapy. Two patients refused further treatment, one was in good condition. In summary, 13/16 patients were normalised following multimodal treatment, 3 patients refused further treatment.

Surgical combined with non-surgical treatment can lead to remission in nearly all patients suffering from recurrent Cushing’s disease. In case of persistence, medical treatment with Rosiglitazone may lead to a temporary normalisation at the least, and combined with radiotherapy, is the best method. Treatment with ketoconazol can be considered only for a transitory improvement of symptoms. Shaped beam radiosurgery may lead to earlier success than conventional radiotherapy. In cases of severe metabolic disturbance bilateral adrenalectomy is still the treatment of choice.