Pituitary (2015) 18:179–180 DOI 10.1007/s11102-015-0647-4
Cushing disease: Where do we stand, where are we heading? Maria Fleseriu • Stephan Petersenn
Ó Springer Science+Business Media New York 2015
Cushing disease is a fascinating disorder, one which poses a great clinical challenge to endocrinologists. Both the screening and confirmatory diagnosis are hindered by overlapping clinical features and biochemical test results in patients with Cushing’s syndrome and other more common morbidities, such as obesity and depression. The search for an underlying pituitary tumor can often be difficult, as an adrenocorticotropic hormone (ACTH)-secreting adenoma may be barely visible on magnetic resonance (MR) imaging, and differential diagnosis of an ectopic ACTH-secreting tumor may require invasive procedures. The surgical management of pituitary adenomas has undergone innovative progress with utilization of an endoscopic endonasal approach. However, even in the hands of the most experienced neurosurgeons, post-operative hypercortisolemia may persist in a large number of patients. Furthermore, there is a remarkably high recurrence rate even after many years of disease remission. Other treatment modalities, such as stereotactic radiosurgery, have also been incorporated into the management paradigm. Progress in the field has been impaired, partly by the rarity of Cushing disease, which has contributed to the lack of large clinical research trials to improve diagnostic procedures and therapeutic techniques. Nevertheless, many decades after Harvey Cushing’s first description, ‘‘The Basophil Adenomas of the Pituitary Body and their Clinical Manifestations: Pituitary Basophilism’’ in 1932, there M. Fleseriu (&) Oregon Health & Science University, CH8N 3303 SW Bond Ave, Portland, OR 97239, USA e-mail: [email protected] S. Petersenn ENDOC Center for Endocrine Tumors, Hamburg, Germany e-mail: [email protected]
have been clear clinical advancements, however more are needed. Clinical guidelines establishing approaches to both the diagnosis and therapeutic treatment of Cushing disease have been published, and large multicenter clinical trials spanning several continents have investigated new approaches to medical treatment. Therefore, the time is ripe to gather current knowledge and understanding of Cushing disease in this Pituitary special issue. Here, we have solicited leading authorities in their respective arenas to provide the most up-to-date information on key areas of disease diagnosis and management, and also controversial topics within the field. As you read the individual articles, you will learn both the basic aspects and the practical approaches to therapy. We are grateful to our colleagues across the world, who have summarized their vast experience, and we hope, that this collection of short and informative articles will spur new ideas and push the innovation of further improvements in the overall care of Cushing disease patients. Towards this goal, and to decrease co-morbidities and improve patient quality of life, a broader understanding of etiology, new input from large patient registries, and longterm follow-up data for all Cushing disease patients, is required. We should remind ourselves that it is not the endocrine tumor itself, but the consequences of cortisol excess, which impose so many burdens on these patients. Caring for Cushing disease patients, while a challenge, is a distinct privilege and we would like to thank the many patients who have participated in clinical research trials. We are confident that improvements in disease screening, diagnosis and long-term care are indeed possible. Our sincere thanks to Shlomo Melmed, Pituitary editorin-chief, contributing authors, and Springer editorial staff for an erudite and timely issue.
123
180
Pituitary (2015) 18:179–180
Maria Fleseriu
123
Stephan Petersenn
Cushing disease: Where do we stand, where are we heading? Maria Fleseriu • Stephan Petersenn
Ó Springer Science+Business Media New York 2015
Cushing disease is a fascinating disorder, one which poses a great clinical challenge to endocrinologists. Both the screening and confirmatory diagnosis are hindered by overlapping clinical features and biochemical test results in patients with Cushing’s syndrome and other more common morbidities, such as obesity and depression. The search for an underlying pituitary tumor can often be difficult, as an adrenocorticotropic hormone (ACTH)-secreting adenoma may be barely visible on magnetic resonance (MR) imaging, and differential diagnosis of an ectopic ACTH-secreting tumor may require invasive procedures. The surgical management of pituitary adenomas has undergone innovative progress with utilization of an endoscopic endonasal approach. However, even in the hands of the most experienced neurosurgeons, post-operative hypercortisolemia may persist in a large number of patients. Furthermore, there is a remarkably high recurrence rate even after many years of disease remission. Other treatment modalities, such as stereotactic radiosurgery, have also been incorporated into the management paradigm. Progress in the field has been impaired, partly by the rarity of Cushing disease, which has contributed to the lack of large clinical research trials to improve diagnostic procedures and therapeutic techniques. Nevertheless, many decades after Harvey Cushing’s first description, ‘‘The Basophil Adenomas of the Pituitary Body and their Clinical Manifestations: Pituitary Basophilism’’ in 1932, there M. Fleseriu (&) Oregon Health & Science University, CH8N 3303 SW Bond Ave, Portland, OR 97239, USA e-mail: [email protected] S. Petersenn ENDOC Center for Endocrine Tumors, Hamburg, Germany e-mail: [email protected]
have been clear clinical advancements, however more are needed. Clinical guidelines establishing approaches to both the diagnosis and therapeutic treatment of Cushing disease have been published, and large multicenter clinical trials spanning several continents have investigated new approaches to medical treatment. Therefore, the time is ripe to gather current knowledge and understanding of Cushing disease in this Pituitary special issue. Here, we have solicited leading authorities in their respective arenas to provide the most up-to-date information on key areas of disease diagnosis and management, and also controversial topics within the field. As you read the individual articles, you will learn both the basic aspects and the practical approaches to therapy. We are grateful to our colleagues across the world, who have summarized their vast experience, and we hope, that this collection of short and informative articles will spur new ideas and push the innovation of further improvements in the overall care of Cushing disease patients. Towards this goal, and to decrease co-morbidities and improve patient quality of life, a broader understanding of etiology, new input from large patient registries, and longterm follow-up data for all Cushing disease patients, is required. We should remind ourselves that it is not the endocrine tumor itself, but the consequences of cortisol excess, which impose so many burdens on these patients. Caring for Cushing disease patients, while a challenge, is a distinct privilege and we would like to thank the many patients who have participated in clinical research trials. We are confident that improvements in disease screening, diagnosis and long-term care are indeed possible. Our sincere thanks to Shlomo Melmed, Pituitary editorin-chief, contributing authors, and Springer editorial staff for an erudite and timely issue.
123
180
Pituitary (2015) 18:179–180
Maria Fleseriu
123
Stephan Petersenn
