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Long-Term Mortality after Transsphenoidal Surgery for Cushing Disease

Brooke Swearingen, MD; Beverly M.K. Biller, MD; Fred G. Barker II, MD; Laurence Katznelson, MD; Steven Grinspoon, MD; Anne Klibanski, MD; and Nicholas T. Zervas, MD
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From Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.

Ann Intern Med. 1999;130(10):821-824. doi:10.7326/0003-4819-130-10-199905180-00015
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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.


Grahic Jump Location
The solid line represents the Kaplan-Meier plot of survival for study patients; the dashed line represents the expected survival rate of an age- and sex-matched sample based on U.S. population data. The standardized mortality ratio was 0.98 (95% CI, 0.44 to 2.2; > 0.2). Numbers indicate patients at risk.
Figure 1. Survival in 159 patients with long-term follow-up who underwent transsphenoidal surgery for Cushing disease compared with survival in the U.S. population.P
Grahic Jump Location
Grahic Jump Location
The solid line represents 125 patients with microadenomas; the dotted line represents 11 patients with macroadenomas. Five- and 10-year cure rates were 96% and 93%, respectively, for patients with microadenomas and 91% and 55% for patients with macroadenomas. The difference in recurrence rates (relative risk, 5.1) was significant ( > 0.03). Numbers indicate patients at risk.
Figure 2. Duration of cure in Cushing disease after transsphenoidal surgery in 136 patients with long-term follow-up, stratified by tumor size.P
Grahic Jump Location




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