Cushing’s Disease is associated with 3-5 fold increase in mortality. The primary treatment modality has been tran-sphenoidal surgery as it allows treatment of the underlying cause without the need for long term hormone replacement. Complete remission rates of 60-85% have been documented, however recurrence can occur in up to 25% of patients postoperatively. In repeat pituitary surgery following recurrence, remission rates are lower at 50-60%, and chances of pituitary failure are higher. Other possible treatment modalities include pituitary radiotherapy, bilateral adrenalectomy and medical management. The latest medication to gain recognition for its role in Cushing’s Disease is Pasireotide, a somatostatin analogue.
This case is a 48 year old lady who underwent pituitary surgery for a non-functioning pituitary cystic lesion. Years later she developed clinical and biochemical features of Cushing’s Disease however imaging studies could not identify an adenoma. Histology from the initial operation was reviewed which showed widespread immunoreactivity for ACTH. She underwent two further pituitary surgeries and achieved remission. Clinically and biochemically she was cured. Unfortunately within a year, the Cushing’s Disease recurred. This is her second recurrence of Cushing’s disease in the context of three previous pituitary surgeries, with no visible lesion on MRI.
This case illustrates the difficult management decisions that need to be made in a patient with recurrent Cushing’s disease. Surgical management has traditionally been considered first line treatment for recurrent Cushing's Disease, however with the recent addition of Pasireotide we need to consider the possibility of a medical alternative which show promise as a viable alternative first line therapy.