Background

Untreated Cushing disease historically has a high mortality rate, but the long-term survival of patients with Cushing disease after transsphenoidal surgery has not been reported.

Objective

To determine long-term mortality rate in patients who are treated for Cushing disease with current management techniques.

Design

Retrospective case series.

Setting

Tertiary care center.

Patients

161 patients (32 men and 129 women; mean age, 38 years) who were treated for Cushing disease between 1978 and 1996.

Intervention

Transsphenoidal adenomectomy and as-needed adjunctive therapy.

Measurement

Record review with follow-up interview.

Results

The cure rate for patients with microadenomas who had no previous therapy was 90% (123 of 137). No perioperative deaths occurred (0 of 193 procedures [95% CI, 0.0% to 1.9%]). Follow-up data (mean, 8.7 years) were obtained for 99% of patients (159 of 161). Six patients died. The 5- and 10-year survival rates were 99% (CI, 97% to 100%) and 93% (CI, 88% to 99%), respectively. Survival was similar to that seen in an age- and sex-matched sample that was based on U.S. population data (standardized mortality ratio, 0.98 [CI, 0.44 to 2.2]; P > 0.2).

Conclusion

Survival of patients treated for Cushing disease with current management techniques between 1978 and 1996 was better than the poor survival historically associated with this disorder.