scholarly journals Treatment Options in Cushing's Disease

2012 ◽  
Vol 6 ◽  
pp. CMO.S6198 ◽  
Author(s):  
Ahmed Rizk ◽  
Juergen Honegger ◽  
Monika Milian ◽  
Tsambika Psaras
Keyword(s):  
Pituitary Adenoma ◽  
Medical Therapy ◽  
Treatment Option ◽  
Cushing’S Disease ◽  
Treatment Options ◽  
Bilateral Adrenalectomy ◽  
Metabolic Effects ◽  
Cushing's Disease ◽  
Line Treatment ◽  
Second Line

Endogenous Cushing's syndrome is a grave disease that requires a multidisciplinary and individualized treatment approach for each patient. Approximately 80% of all patients harbour a corticotroph pituitary adenoma (Cushing's disease) with excessive secretion of adrenocorticotropin-hormone (ACTH) and, consecutively, cortisol. The goals of treatment include normalization of hormone excess, long-term disease control and the reversal of comorbidities caused by the underlying pathology. The treatment of choice is neurosurgical tumour removal of the pituitary adenoma. Second-line treatments include medical therapy, bilateral adrenalectomy and radiation therapy. Drug treatment modalities target at the hypothalamic/pituitary level, the adrenal gland and at the glucocorticoid receptor level and are commonly used in patients in whom surgery has failed. Bilateral adrenalectomy is the second-line treatment for persistent hypercortisolism that offers immediate control of hypercortisolism. However, this treatment option requires a careful individualized evaluation, since it has the disadvantage of permanent hypoadrenalism which requires lifelong glucocorticoid and mineralocorticoid replacement therapy and bears the risk of developing Nelson's syndrome. Although there are some very promising medical therapy options it clearly remains a second-line treatment option. However, there are numerous circumstances where medical management of CD is indicated. Medical therapy is frequently used in cases with severe hypercortisolism before surgery in order to control the metabolic effects and help reduce the anestesiological risk. Additionally, it can help to bridge the time gap until radiotherapy takes effect. The aim of this review is to analyze and present current treatment options in Cushing's disease.

scholarly journals Radiotherapy as a tool for the treatment of Cushing’s disease

2019 ◽  
Vol 180 (5) ◽  
pp. D9-D18 ◽  
Author(s):  
Frederic Castinetti ◽  
Thierry Brue ◽  
Oskar Ragnarsson
Keyword(s):  
Medical Treatment ◽  
Cushing’S Disease ◽  
Pituitary Surgery ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease ◽  
Limiting Factor ◽  
Pituitary Insufficiency ◽  
Line Treatment ◽  
Second Line ◽  
High Prevalence

Treatment of Cushing’s disease (CD) is one of the most challenging tasks in endocrinology. The first-line treatment, transsphenoidal pituitary surgery, is associated with a high failure rate and a high prevalence of recurrence. Re-operation is associated with an even higher rate of a failure and recurrence. There are three main second-line treatments for CD – pituitary radiation therapy (RT), bilateral adrenalectomy and chronic cortisol-lowering medical treatment. All these treatments have their limitations. While bilateral adrenalectomy provides permanent cure of the hypercortisolism in all patients, the unavoidable chronic adrenal insufficiency and the risk of development of Nelson syndrome are of concern. Chronic cortisol-lowering medical treatment is not efficient in all patients and side effects are often a limiting factor. RT is efficient for approximately two-thirds of all patients with CD. However, the high prevalence of pituitary insufficiency is of concern as well as potential optic nerve damage, development of cerebrovascular disease and secondary brain tumours. Thus, when it comes to decide appropriate treatment for patients with CD, who have either failed to achieve remission with pituitary surgery, or patients with recurrence, the pros and cons of all second-line treatment options must be considered.

scholarly journals Recurrence after pituitary surgery in adult Cushing’s disease: a systematic review on diagnosis and treatment

Endocrine ◽  
2020 ◽  
Vol 70 (2) ◽  
pp. 218-231
Author(s):  
Leah T. Braun ◽  
German Rubinstein ◽  
Stephanie Zopp ◽  
Frederick Vogel ◽  
Christine Schmid-Tannwald ◽  
...  
Keyword(s):  
Medical Therapy ◽  
Cushing’S Disease ◽  
Small Sample Size ◽  
Case Reports ◽  
Treatment Options ◽  
Pituitary Surgery ◽  
Small Sample ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease ◽  
Success Rates

Abstract Purpose Recurrence after pituitary surgery in Cushing’s disease (CD) is a common problem ranging from 5% (minimum) to 50% (maximum) after initially successful surgery, respectively. In this review, we give an overview of the current literature regarding prevalence, diagnosis, and therapeutic options of recurrent CD. Methods We systematically screened the literature regarding recurrent and persistent Cushing’s disease using the MESH term Cushing’s disease and recurrence. Of 717 results in PubMed, all manuscripts in English and German published between 1980 and April 2020 were screened. Case reports, comments, publications focusing on pediatric CD or CD in veterinary disciplines or studies with very small sample size (patient number < 10) were excluded. Also, papers on CD in pregnancy were not included in this review. Results and conclusions Because of the high incidence of recurrence in CD, annual clinical and biochemical follow-up is paramount. 50% of recurrences occur during the first 50 months after first surgery. In case of recurrence, treatment options include second surgery, pituitary radiation, targeted medical therapy to control hypercortisolism, and bilateral adrenalectomy. Success rates of all these treatment options vary between 25 (some of the medical therapy) and 100% (bilateral adrenalectomy). All treatment options have specific advantages, limitations, and side effects. Therefore, treatment decisions have to be individualized according to the specific needs of the patient.

scholarly journals Clinical and Endocrine Responses to Pituitary Radiotherapy in Pediatric Cushing’s Disease: An Effective Second-Line Treatment

2003 ◽  
Vol 88 (1) ◽  
pp. 34-37 ◽  
Author(s):  
Helen L. Storr ◽  
P. Nicholas Plowman ◽  
Paul V. Carroll ◽  
Inge François ◽  
Gerasimos E. Krassas ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Line Treatment ◽  
Second Line

scholarly journals Bilateral adrenalectomy in Cushing's disease: Altered long-term quality of life compared to other treatment options

2019 ◽  
Vol 80 (1) ◽  
pp. 32-37 ◽  
Author(s):  
Pauline Sarkis ◽  
Muriel Rabilloud ◽  
Jean-Christophe Lifante ◽  
Anna Siamand ◽  
Emmanuel Jouanneau ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Cushing’S Disease ◽  
Treatment Options ◽  
Bilateral Adrenalectomy ◽  
Cushing's Disease

scholarly journals A real-world analysis of second-line treatment options in pancreatic cancer: liposomal-irinotecan plus 5-fluorouracil and folinic acid

2019 ◽  
Vol 11 ◽  
pp. 175883591985319 ◽  
Author(s):  
Markus Kieler ◽  
Matthias Unseld ◽  
Daniela Bianconi ◽  
Werner Scheithauer ◽  
Gerald W. Prager
Keyword(s):  
Treatment Option ◽  
Real World ◽  
Treatment Options ◽  
Progression Free Survival ◽  
Line Treatment ◽  
Second Line ◽  
Real World Evidence ◽  
Nanoliposomal Irinotecan ◽  
Liposomal Irinotecan ◽  
Line Setting

Background: Nanoliposomal irinotecan (nal-IRI) plus 5-fluorouracil/leucovorin (5-FU/LV) is a novel treatment option for gemcitabine-pretreated metastatic pancreatic adenocarcinoma (PAC) patients, but real-world evidence is rare. Our aim was to determine the effectiveness and tolerability of this regimen in advanced PAC patients and to compare it with oxaliplatin plus fluoropyrimidines in the second-line setting after failure of gemcitabine. Methods: This is a retrospective single-center analysis of all patients who have been treated with nal-IRI plus 5-FU/LV. To compare its effectiveness with other second-line treatment options, all patients who had received oxaliplatin plus fluoropyrimidines after gemcitabine-based chemotherapy were also eligible for analysis. Results: Fifty-two patients were treated with nal-IRI plus 5-FU/LV between April 2016 and August 2018. The median progression-free survival (PFS) was 3.84 months and the median overall survival (OS) was 6.79 months. Median OS from the beginning of the treatment for advanced disease was 19.9 months. Median PFS in patients that received nal-IRI plus 5-FU/LV as second-line treatment after gemcitabine-based chemotherapy was 4.49 months whereas median PFS in a matched cohort of patients treated with oxaliplatin plus fluoropyrimidines was 3.44 months ( p = 0.007). Between these two groups the median OS of patients with CA 19-9 levels above the statistical median (⩾772.8 kU/l) differed significantly (9.33 versus 6.18 months, p = 0.038). Conclusion: Our data confirms the effectiveness of nal-IRI plus 5-FU/LV treatment as a well-tolerated regimen in the treatment of advanced PAC and extends available data on its use as a second-line treatment option when compared with oxaliplatin plus fluoropyrimidines.

scholarly journals Favorable long-term outcomes of bilateral adrenalectomy in Cushing's disease

2014 ◽  
Vol 171 (2) ◽  
pp. 209-215 ◽  
Author(s):  
Andrea Oßwald ◽  
Eva Plomer ◽  
Christina Dimopoulou ◽  
Monika Milian ◽  
Rainer Blaser ◽  
...  
Keyword(s):  
Treatment Option ◽  
Cushing’S Disease ◽  
Bilateral Adrenalectomy ◽  
Morbidity And Mortality ◽  
Cushing's Disease ◽  
Healthy Population ◽  
Long Term Outcomes ◽  
Surgical Morbidity

ObjectiveBilateral adrenalectomy (BADX) is an important treatment option for patients with Cushing's syndrome (CS). Our aim is to analyze the long-term outcomes, surgical, biochemical, and clinical as well as morbidity and mortality, of patients who underwent BADX.DesignA total of 50 patients who underwent BADX since 1990 in two German centers were identified. Of them, 34 patients had Cushing's disease (CD), nine ectopic CS (ECS), and seven ACTH-independent bilateral adrenal hyperplasia (BAH).MethodsStandardized follow-up examination was performed in 36 patients with a minimum follow-up time of 6 months after BADX and a median follow-up time of 11 years.ResultsSurgical morbidity and mortality were 6 and 4% respectively. All patients were found to be in remission after BADX. Almost all Cushing's-specific comorbidities except for psychiatric diseases improved significantly. Health-related quality of life remained impaired in 45.0% of female and 16.7% of male patients compared with a healthy population. The median number of adrenal crises per 100 patient-years was four. Nelson tumor occurred in 24% of CD patients after a median time span of 51 months. Long-term mortality after 10 years was high in ECS (44%) compared with CD (3%) and BAH (14%).ConclusionsBADX is an effective and relatively safe treatment option especially in patients with CD. The majority of patients experience considerable improvement of Cushing's symptoms.

scholarly journals The Treatment of Cushing's Disease

2015 ◽  
Vol 36 (4) ◽  
pp. 385-486 ◽  
Author(s):  
Rosario Pivonello ◽  
Monica De Leo ◽  
Alessia Cozzolino ◽  
Annamaria Colao
Keyword(s):  
Medical Therapy ◽  
Cushing’S Syndrome ◽  
Cushing’S Disease ◽  
Pituitary Tumor ◽  
Cushing's Syndrome ◽  
Cushing's Disease ◽  
Primary Therapy ◽  
Line Treatment ◽  
Adrenal Surgery ◽  
Disease Remission

Abstract Cushing's disease (CD), or pituitary-dependent Cushing's syndrome, is a severe endocrine disease caused by a corticotroph pituitary tumor and associated with increased morbidity and mortality. The first-line treatment for CD is pituitary surgery, which is followed by disease remission in around 78% and relapse in around 13% of patients during the 10-year period after surgery, so that nearly one third of patients experience in the long-term a failure of surgery and require an additional second-line treatment. Patients with persistent or recurrent CD require additional treatments, including pituitary radiotherapy, adrenal surgery, and/or medical therapy. Pituitary radiotherapy is effective in controlling cortisol excess in a large percentage of patients, but it is associated with a considerable risk of hypopituitarism. Adrenal surgery is followed by a rapid and definitive control of cortisol excess in nearly all patients, but it induces adrenal insufficiency. Medical therapy has recently acquired a more important role compared to the past, due to the recent employment of novel compounds able to control cortisol secretion or action. Currently, medical therapy is used as a presurgical treatment, particularly for severe disease; or as postsurgical treatment, in cases of failure or incomplete surgical tumor resection; or as bridging therapy before, during, and after radiotherapy while waiting for disease control; or, in selected cases, as primary therapy, mainly when surgery is not an option. The adrenal-directed drug ketoconazole is the most commonly used drug, mainly because of its rapid action, whereas the glucocorticoid receptor antagonist, mifepristone, is highly effective in controlling clinical comorbidities, mainly glucose intolerance, thus being a useful treatment for CD when it is associated with diabetes mellitus. Pituitary-directed drugs have the advantage of acting at the site responsible for CD, the pituitary tumor. Among this group of drugs, the dopamine agonist cabergoline and the somatostatin analog pasireotide result in disease remission in a consistent subgroup of patients with CD. Recently, pasireotide has been approved for the treatment of CD when surgery has failed or when surgery is not an option, and mifepristone has been approved for the treatment of Cushing's syndrome when associated with impairment of glucose metabolism in case of the lack of a surgical indication. Recent experience suggests that the combination of different drugs may be able to control cortisol excess in a great majority of patients with CD.

scholarly journals Severe hepatocytolisis syndrome - a challenge in medical therapy of Cushing’s disease

2015 ◽  
Vol 21 (3) ◽  
pp. 132-136
Author(s):  
Ana Valea ◽  
Mara Carsote ◽  
Adina Ghemigian ◽  
Andra Morar ◽  
D.P. Dumitru ◽  
...  
Keyword(s):  
Pituitary Adenoma ◽  
Medical Therapy ◽  
Cushing’S Disease ◽  
Tumor Resection ◽  
Clinical Manifestations ◽  
Cushing's Disease ◽  
Technical Equipment ◽  
Acth Secretion ◽  
Surgery Outcome ◽  
Disease Case

ABSTRACT Introduction Cushing’s disease is a rare endocrine disorder characterized by persistent hypercortisolism due to excessive, autonomous ACTH secretion by a pituitary adenoma. Transsphenoidal adenomectomy is the main therapeutic option in Cushing’s disease. When imaging studies are unable to demonstrate an obvious pituitary mass complete tumor resection is difficult and often encumbered by risk of relapse. Medical therapy is a second-line option or the first-line treatment for patients with high surgical risk, incomplete surgical resection or relapse. Rarely classic clinical manifestations limit the selection of medical therapy for Cushing’s disease. Case presentation We present the case of a male patient with Cushing’s disease complicated with diabetes mellitus and severe hepatocytolisis syndrome with no visible pituitary adenoma on MRI studies. In the absence of technical equipment petrosal sinus sampling could not be performed in order to improve surgery outcome Conclusion The presence of unbalanced diabetes and severe hepatocytolisis syndrome made it impossible to use steroidogenesis inhibitors (mitotane, ketoconazole, metyrapone, etomidate) or inhibitors of ACTH secretion as pasireotide that represent a new option in achieving control of hypercortisolism

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