Objective: The past 2 decades have seen advances in the surgical management of Cushings disease (CD). Our aim was to meet the need for current data on clinical features, long-term outcomes, and prognostic indicators.
Patients and methods: We conducted a retrospective study of 71 patients treated by trans-sphenoidal surgery (TSS) for CD. All patients were operated on by the same surgeon in a single centre between 19882009. Diagnosis was confirmed using Low and high dose-DSTs, and CRH tests, with selected patients undergoing IPSS. 58 patients underwent microscopic-TSS at first surgery, whereas 13 had Endoscopic-TSS.
Results: Median follow up was 50 months (IQR 22115 months). Median age at diagnosis was 39 years (IQR 3050 years). Male:female ratio was 1:3.4. Follow up data were available on 67/71 patients. 82% (55/67) achieved initial clinical remission, of which 8 suffered disease recurrence. Mean time to recurrence was 3.9 years (IQR 3 months-14 years). 3 outcome groups were identified: Long-term Cure 70% (47/67), Persistent Disease 18% (12/67), and Recurrent Disease 12% (8/67).
Long-term cure rates were higher in patients with undetectable (<30 nmol/l) post-operative cortisol levels (87%). They were also increased in patients with histology positive for ACTH-containing adenomas (82%), compared to those with negative histology (57%). Further treatment for patients with recurrent/persistent disease included revision TSS (n=11), Radiotherapy (n=7), and Adrenalectomy (n=7). Common complications following TSS were transient DI (60%), and CSF leak (16%). Hypopituitarism was present in 81% of patients at final follow up. Mortality was increased in persistent/recurrent disease, compared to the long-term cured (P=0.04). 4 deaths were documented (2 recurrent, 1 persistent disease, 1 cured 20 years previously).
Conclusion: Our favourable remission/cure rates, serve to underline the importance of an experienced surgeon in the management of CD. Favourable prognostic factors include undetectable post-operative cortisols and ACTH-positive histology. The reduced mortality in the long-term cured, demonstrates the importance of aggressive treatment of CD.