From the Guidelines
Cushing's disease can be surgically corrected, with selective adenomectomy being the first-line treatment of choice, as recommended by the consensus guideline for the diagnosis and management of pituitary adenomas in childhood and adolescence. This approach involves the selective removal of the adenoma, performed by a surgeon experienced in pediatric transsphenoidal surgery, with the goal of maximizing the potential for normal pituitary tissue to remain in situ 1. The optimal treatment for Cushing's disease is surgical resection, which has been shown to have low rates of post-operative hypopituitarism in several large studies in children and adolescents 1.
Some key points to consider when evaluating surgical correction of Cushing's disease include:
- The importance of surgeon experience in predicting success, as selective microadenomectomy can be technically very difficult in children 1
- The association of early post-operative remission with identification of the adenoma at surgery, and long-term remission with factors such as younger age, smaller adenoma, and absence of cavernous sinus or dural invasion 1
- The potential for repeat surgery to result in early biochemical remission, with a reported success rate of 93% in 27 patients 1
- The need for lifelong follow-up for children treated for Cushing's disease, due to the risk of recurrence up to 15 years after apparent surgical cure 1
Overall, surgical correction of Cushing's disease is a viable and effective treatment option, with a strong recommendation for selective adenomectomy as the first-line treatment of choice 1.
From the FDA Drug Label
1 INDICATIONS AND USAGE 1.1 Cushing's Disease
SIGNIFOR is indicated for the treatment of adult patients with Cushing's disease for whom pituitary surgery is not an option or has not been curative.
Cushing's disease can be surgically corrected, but the provided drug label indicates that the medication is intended for patients for whom pituitary surgery is not an option or has not been curative 2. This implies that surgery is a potential treatment option for some patients with Cushing's disease.
From the Research
Surgical Correction of Cushing Disease
- Cushing disease can be surgically corrected using transsphenoidal surgery (TSS) 3, 4, 5, 6, 7.
- The goal of TSS is to remove the adrenocorticotropic hormone (ACTH)-producing tumor in the pituitary gland, which is the primary cause of Cushing disease.
- Studies have shown that TSS can be an effective treatment for Cushing disease, with remission rates ranging from 70% to 91% after one operation 3, 4, 5.
Factors Affecting Surgical Outcome
- The success of TSS depends on careful preoperative evaluation, including magnetic resonance imaging (MRI) and inferior petrosal sinus (IPS) sampling 3.
- The surgeon's experience and the use of selective adenomectomy can also impact the outcome of the surgery 3, 4.
- The size of the tumor does not seem to affect the remission rate, with similar remission rates observed for microadenomas and macroadenomas 4.
Postoperative Care and Follow-up
- Postoperative care and follow-up are crucial to monitor for disease recurrence and to manage potential complications, such as pituitary hormone insufficiencies 3, 4, 7.
- Early postoperative hypocortisolism can be a predictor of complete tumor removal and high rates of remission 5.
- Long-term postoperative endocrinological follow-up can facilitate early detection of recurrent hypercortisolemia and improve patient outcomes 7.
Challenges and Solutions
- Despite the effectiveness of TSS, persistent Cushing disease can occur in around 22% of patients due to incomplete tumor resection or misdiagnosis 6.
- A multidisciplinary approach is needed to minimize potential pitfalls during diagnosis and treatment, and to provide the best care for patients who have undergone unsuccessful surgery 6.