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.