Therapeutic options after surgical failure in Cushing's disease: A critical review

Rubinstein, German; Oßwald, Andrea; Zopp, Stephanie; Ritzel, Katrin; Theodoropoulou, Marily; Beuschlein, Felix; Reincke, Martín

doi:10.1016/j.beem.2019.04.004

Cited by 25 publications

(20 citation statements)

References 61 publications

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“…Remission rate in persistent disease is about 54% while remission rates are higher (64%) in recurrent disease. A thorough review of outcomes in persistent versus recurrent CD can be found in Rubinstein et al [54]. Pituitary surgery should be conducted in a center with high experience (more than 25 surgeries per year [55])-this applies both for persistent, recurrent, and surgery-naïve CD as well.…”

Section: Second Pituitary Surgery (Tss)mentioning

confidence: 99%

“…Pituitary surgery should be conducted in a center with high experience (more than 25 surgeries per year [ 55 ])—this applies both for persistent, recurrent, and surgery-naïve CD as well. A second pituitary surgery seems to be currently the first-line therapy in recurrent CD with a mean remission rate of 64%, but high rates of variability (38–90%) is reported in the literature [ 54 ]. Success rates seem to be lower than in first surgery [ 56 ].…”

Section: Treatment Options After Recurrence and In Persistent Cushingmentioning

confidence: 99%

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

et al. 2020

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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.

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Section: Second Pituitary Surgery (Tss)mentioning

confidence: 99%

Section: Treatment Options After Recurrence and In Persistent Cushingmentioning

confidence: 99%

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

et al. 2020

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“…Recently, Koulouri et al [206] reported the usefulness of 11 C-methionine PET co-registered with MRI for detecting residual tumors in cases of persistent acromegaly, which could be differentiated on MRI (Figure 7). Moreover, tumor fibrosis and cavernous sinus invasion are more common in repeat surgery [145,207]. Neurosurgeons tend to perform more extensive surgery during repeat TSS, aiming for total or hemihypophysectomy, especially when no selective tumorectomy is possible after an exploratory approach is used.…”

Section: Treatmentmentioning

confidence: 99%

Cushing’s Disease

Nishioka

Yamada²

2019

JCM

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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.

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“…The treatment of choice is transsphenoidal surgery (TSS) with selective removal of the adenoma tissue. Rates for persistence of CD or recurrence after initial remission were reported with a great variability depending on the ratio of micro-/macroadenoma, the experience of the surgeons and the definition for persistence and recurrence (1,2). Based on meta-analyses the rates for persistence and recurrence after initial TSS ranged from 22 to 24% (persistence) (3,4,5) and 10-12% (recurrence) (4), respectively.…”

Section: Introductionmentioning

confidence: 99%

Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations

Reincke

Albani

Assié

et al. 2021

European Journal of Endocrinology

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BACKGROUND: Corticotroph tumor progression (CTP) leading to Nelson’s syndrome (NS) is a severe and difficult-to-treat complication subsequent to bilateral adrenalectomy (BADX) for Cushing’s disease. Its characteristics are not well described, and consensus recommendations for diagnosis and treatment are missing. METHODS: A systematic literature search was performed focusing on clinical studies and case series (≥5 patients). Definition, incidence, treatment and long-term outcomes of CTP/NS after BADX were analyzed using descriptive statistics. The results were presented and discussed at an interdisciplinary consensus workshop attended by international pituitary experts in Munich on October 28th, 2018. RESULTS: Data covered definition and incidence (34 studies, 1275 patients), surgical outcome (12 studies, 187 patients), outcome of radiation therapy (21 studies, 273 patients), and medical therapy (15 studies, 72 patients). CONCLUSIONS: We endorse the definition of CTP-BADX/NS as radiological progression or new detection of a pituitary tumor on thin-section MRI. We recommend surveillance by MRI after 3 months and every 12 months for the first 3 years after BADX. Subsequently, we suggest clinical evaluation every 12 months and MRI at increasing intervals every 2-4 years (depending on ACTH and clinical parameters). We recommend pituitary surgery as first-line therapy in patients with CTP-BADX/NS. Surgery should be performed before extrasellar expansion of the tumor to obtain complete and long-term remission. Conventional radiotherapy or stereotactic radiosurgery should be utilized as second-line treatment for remnant tumor tissue showing extrasellar extension.

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Therapeutic options after surgical failure in Cushing's disease: A critical review

Cited by 25 publications

References 61 publications

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

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

Cushing’s Disease

Corticotroph tumor progression after bilateral adrenalectomy (Nelson’s syndrome): systematic review and expert consensus recommendations

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