Abstract
In patients with Cushing’s disease (CD), prompt diagnosis and treatment are essential for favorable long-term outcomes, although this remains a challenging task. The differential diagnosis of CD is still difficult in some patients, even with an organized stepwise diagnostic approach. Moreover, despite the use of high-resolution magnetic resonance imaging (MRI) combined with advanced fine sequences, some tumors remain invisible. Surgery, using various surgical approaches for safe maximum tumor removal, still remains the first-line treatment for most patients with CD. Persistent or recurrent CD after unsuccessful surgery requires further treatment, including repeat surgery, medical therapy, radiotherapy, or sometimes, bilateral adrenalectomy. These treatments have their own advantages and disadvantages. However, the most important thing is that this complex disease should be managed by a multidisciplinary team with collaborating experts. In addition, a personalized and individual-based approach is paramount to achieve high success rates while minimizing the occurrence of adverse events and improving the patients’ quality of life. Finally, the recent new insights into the pathophysiology of CD at the molecular level are highly anticipated to lead to the introduction of more accurate diagnostic tests and efficacious therapies for this devastating disease in the near future.
Highlights
A significant burden has been demonstrated in patients with Cushing’s disease (CD) due to comorbidities, increased mortality, and impaired health-related quality of life (HRQoL)
Recent evidence indicates that following the long-term exposure to excess glucocorticoids, these burdens persist, at least partially, after CD has been “cured.” many investigators have emphasized the crucial importance of rapid diagnosis and treatment as well as appropriate follow-up
Repeat surgery should only be considered in patients in whom a distinct and accessible tumor is visible on magnetic resonance imaging (MRI), but it is usually associated with a relatively higher rate of failure and recurrence
Summary
Cushing reported 12 patients with a serious metabolic disorder, which he ascribed to pituitary basophilic tumors [1]. The term Cushing’s syndrome (CS) refers to the clinical symptoms and signs of inappropriately elevated levels of plasma glucocorticoids. CD refers to a rare disorder caused by pituitary corticotroph tumors and represents the most common cause (nearly 70% in adults) of endogenous CS. Prolonged systemic exposure to elevated cortisol levels results in a significant clinical burden in patients with CD due to comorbidities, increased mortality, and impaired health-related quality of life (HRQoL). Recent studies have clearly demonstrated the impact of early accurate diagnosis and treatment on long-term outcomes [2,3,4,5,6,7]. Despite significant progress, the diagnosis and treatment of CD remains challenging, and comprehensive endocrine management is often required
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