Cushing's disease: Results of treatment and factors affecting outcome

Ammini, Ariachery C.; Bhattacharya, Saptarshi; Sahoo, Jaya Praksh; Philip, Jim; Tandon, Nikhil; Goswami, Ravinder; Jyotsna, Viveka P; Khadgawat, Rajesh; Chumber, Sunil; Seth, A.; Karak, Asis Kumar; Sharma, Bhawani S.; Chandra, P. Sarat; Suri, Ashish; Sharma, Manish; Kale, Shashank S.; Singh, Manmohan

doi:10.14310/horm.2002.1312

Cited by 30 publications

(12 citation statements)

References 49 publications

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“…This is not especially surprising, as microadenomas are often not visible on MRI, whereas pituitary incidentalomas are found in 10% of the normal population (45). Thus, regarding factors predictive of a good outcome, the presence of a microadenoma and subsequent positive histology are strong indicators of successful surgery, in accord with other studies (46), but this is in contrast to positive imaging (14) and is unrelated to the cyclic presence of hypercortisolaemia. As expected, the remission rate was higher in microadenomas compared with macroadenomas, as previously reported (12,20,21,47,48,49).…”

Section: Remission Ratessupporting

confidence: 61%

Long-term remission and recurrence rates in Cushing's disease: predictive factors in a single-centre study

Alexandraki

Kaltsas

Isidori

et al. 2013

European Journal of Endocrinology

171

123

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Objective: To investigate the early and late outcomes of patients with Cushing's disease (CD) submitted to a neurosurgical procedure as first-line treatment. Design: In this single-centre retrospective case notes study, 131 patients with CD with a minimum follow-up period of 6 years (124 operated by transsphenoidal surgery (TSS) and seven by the transcranial approach) were studied. Apparent immediate cure: post-operative 0900 h serum cortisol level !50 nmol/l; remission: cortisol insufficiency or restoration of 'normal' cortisol levels with resolution of clinical features; and recurrence: dexamethasone resistance and relapse of hypercortisolaemic features. Results: In patients operated by TSS, remission of hypercortisolaemia was found in 72.8% of 103 microadenomas and 42.9% of 21 macroadenomas, with recurrence rates 22.7 and 33.3% respectively with a 15-year mean follow-up (range, 6-29 years). Of 27 patients with microadenomas operated after 1991, with positive imaging and pathology, 93% obtained remission with 12% recurrence. In multivariate analysis, the time needed to achieve recovery of hypothalamo-pituitary-adrenal axis was the only significant predictor of recurrence; all patients who recurred showed recovery within 3 years from surgery: 31.3% of patients had total hypophysectomy with no recurrence; 42% of patients with selective adenomectomy and 26.5% with hemi-hypophysectomy showed recurrence rates of 31 and 13% respectively (c 2 Z6.275, PZ0.03). Strict remission criteria were not superior in terms of the probability of recurrence compared with post-operative normocortisolaemia. Conclusions: Lifelong follow-up for patients with CD appears essential, particularly for patients who have shown rapid recovery of their axis. The strict criteria previously used for 'apparent cure' do not appear to necessarily predict a lower recurrence rate.

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Section: Remission Ratessupporting

confidence: 61%

Long-term remission and recurrence rates in Cushing's disease: predictive factors in a single-centre study

Alexandraki

Kaltsas

Isidori

et al. 2013

European Journal of Endocrinology

171

123

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“…Part of this is published. [17] Among the 40 patients with adrenal CS, 23 were adrenal carcinomas, 15 were adrenal adenoma, one was AIMAH and one was PPNAD.…”

Section: Resultsmentioning

confidence: 99%

Etiology and clinical profile of patients with Cushing′s syndrome: A single center experience

Ammini

Tandon

Gupta

et al. 2014

Indian J Endocr Metab

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Background:There is little published literature on the profile of patients with Cushing's syndrome (CS) from India. The aim of this study was to compile data of CS patients treated at this hospital.Materials and Methods:Patients referred to the endocrine services of this hospital for diagnosis/treatment of CS from January 1985 to July 2012 were the subjects for this study. All patients had detailed medical history, physical examination and biochemical and hormonal assays (which changed with availability of tests and changing views). Assays for plasma adrenocorticotropic hormone (ACTH) (late 90s), salivary cortisol estimation, IJV sampling for ACTH and corticotrophin releasing hormone stimulation tests were added on later. Imaging included computed tomography (CT), magnetic resonance imaging (since the late 80's) and 68Ga DOTA-TOC/FDG PET-CT (2008).Results:Three hundred sixty-four patients (250 females, 114 males, age 6 months to 65 years, mean 28 years + 12 years) were diagnosed to have CS during this period. Two hundred and ninety-three patients (80.5%) were ACTH dependent (CD 215, ectopic ACTH syndrome 22, occult ACTH source 56) while 71 (19.5%) were ACTH independent (adrenal carcinoma 36, adenoma 30, primary pigmented nodular adrenal disease 4, AIMAH 1). Pituitary macro adenoma was seen in 14% of the CD cases. The most common presenting complaints were hypertension and diabetes mellitus. A total of 63% patients complained of weight gain while 15% had lost weight. Myopathy, infections, skeletal fractures and psychiatric problems were the other common observations in our patients.Conclusion:The clinical spectrum was broad. CD was the most common cause for CS.

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“…Patient and tumor features. Male gender has been associated with increased tumor aggressiveness, resulting in poorer surgical results (14,132,234). A worse prognosis has been associated with large macroadenomas because the presence of a tumor with a diameter of 2.0 cm or more has been demonstrated to be associated with an increased likelihood (odds ratio, 35) of residual disease after surgery (112).…”

Section: Predictive Factors For the Outcome Of Pituitary Surgerymentioning

confidence: 99%

“…In seven series including only patients who underwent selective adenomectomy, no case of new pituitary deficiency was described (93,100,123,130,132,138,168), but in all series that included only patients who had undergone total hypophysectomy, hypopituitarism was always reported (91,92,99,101). Considering all the studies reporting the general rate of hypopituitarism, this rate ranges between 0.9 and 93.3% (mean, 29.6%; median, 17.6%) (14,18,91,107,117,126,128,133,134,137,139,154,157,160,163,166,170,171).…”

Section: Hypopituitarismmentioning

confidence: 99%

The Treatment of Cushing's Disease

et al. 2015

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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.

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Cushing's disease: Results of treatment and factors affecting outcome

Cited by 30 publications

References 49 publications

Long-term remission and recurrence rates in Cushing's disease: predictive factors in a single-centre study

Long-term remission and recurrence rates in Cushing's disease: predictive factors in a single-centre study

Etiology and clinical profile of patients with Cushing′s syndrome: A single center experience

The Treatment of Cushing's Disease

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