Diagnosis and treatment of Cushing's disease without remission after transsphenoidal surgery

Abstract

Objective To discuss the diagnosis and treatment of Cushing's disease without remission after transsphenoidal surgery. Methods 12 patients of Cushing's disease without remission after transsphenoidal surgery were investigated in this retrospective study. There were 2 males and 10 females with an average age of 36(21-46)years old. Cushing's disease was diagnosed with the typical symptoms and signs of Cushing's syndrome, endocrinological examination and CT or MRI. Transsphenoidal surgery was performed in all patients. None of the patients achieved remission after operation. Serum and urinary free cortisol(UFC)were still elevated 3 months after operation. Subtotal adrenalectomy combined with pituitary radiotherapy were performed in 5 patients, single subtotal adrenalectomy in 4, pituitary radiotherapy in 2 and bilateral adrenalectomy with adrenal autotransplantation in 1 patient. Results Those receiving subtotal adrenalectomy combined with pituitary radiotherapy had no recurrence or Nelson's syndrome during 20 years of follow-up, but 4 patients receiving single subtotal adrenalectomy recurred after 2 to 9 years after operation. 2 patients achieved remission with pituitary radiotherapy 1 to 2 years later without recurrence during 5 years of follow-up. One patient who underwent bilateral adrenalectomy with adrenal autotransplantation needed steroid replacement and Nelson's syndrome occurred during 2 years of follow-up because transplanted adrenal gland had no function. Conclusion The diagnosis of Cushing's disease should be reconfirmed for patients without remission after transsphenoidal surgery and the treatment should be individualized based on the results of operation, pathology and imaging. Key words: Cushing's disease; Transsphenoidal surgery