Sporadic Zollinger-Ellison syndrome with ectopic production of corticotropin: surgical management.

OBJECTIVE

To describe two patients with concurrent Zollinger-Ellison syndrome and ectopic production of corticotropin in whom Cushing's syndrome was managed surgically.

METHODS

Two case vignettes are presented, and a general approach is discussed for determining a management strategy for optimal potential for survival.

RESULTS

The prognosis associated with medical management of patients with sporadic Zollinger-Ellison syndrome and Cushing's syndrome attributable to ectopic production of adrenocorticotropic hormone (corticotropin) is dismal. Two surgical options may yield improved outcomes. The first approach is bilateral adrenalectomy followed by replacement therapy with corticosteroids and mineralocorticoids. The second surgical approach consists of removal of the organ producing the corticotropin (the liver) and performance of hepatic transplantation. These two treatment strategies were used in our two patients, both of whom had widely metastatic disease at the time of initial assessment. The patient who underwent bilateral adrenalectomy continued to do well 4 years postoperatively.

CONCLUSION

Treatment of patients with Zollinger-Ellison syndrome and ectopic production of corticotropin presents a challenge. Because results with medical therapy have been suboptimal, aggressive surgical intervention seems warranted.