Results of Gamma Knife surgery for Cushing's disease

Sheehan, Jason P.; Xu, Zhiyuan; Salvetti, David J.; Schmitt, Paul J.; Vance, Mary Lee

doi:10.3171/2013.7.jns13217

Cited by 89 publications

(79 citation statements)

References 44 publications

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“…Considering the studies on GK, in a follow-up period of 2-264 months (mean, 48.9 mo; median, 46.1 mo), the remission rate was 10 -100% (mean, 58.7%; median, 55.6%) (318 -321, 323-334, 336, 337, 339 -347). The time to remission is nonhomogeneously expressed in the different studies, being reported as a range (1-166 mo) in 10 studies (318, 327-329, 331, 334, 342-344, 346), as a mean (3-58.3 mo) in 11 studies (323, 327-329, 332, 336, 337, 342-344, 347) and as a median (14 -45 mo) in five studies (329,334,341,342,346). In these series, the recurrence rate was 0 -100%, with mean and median re- (322,335,338,339,349).…”

Section: Efficacy Of Pituitary Radiotherapymentioning

confidence: 96%

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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Section: Efficacy Of Pituitary Radiotherapymentioning

confidence: 96%

The Treatment of Cushing's Disease

et al. 2015

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“…The optimal dose is 20-25 Gy for SRS and 45-50.4 Gy given over 5 weeks for radiation therapy. 85,98 Generally, the incidence of remission in CD seems to be similar between the various forms of radiosurgery (43%-58%) and fractionated therapies (46%-84%; Table 3), 13,30,85,94,98,108,109 but the pace of the therapeutic response may be faster with SRS. The major risk of SRS or fractionated irradiation therapy is loss of pituitary function, which is presumably the net effect of surgery and radiation.…”

Section: Radiation Therapymentioning

confidence: 99%

Cushing's disease: pathobiology, diagnosis, and management

Lonser

Nieman

Oldfield

2017

JNS

150

114

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Cushing's syndrome (CS) is caused by prolonged supraphysiological levels of circulating cortisol. Cushing's disease (CD) is the most common etiology (70%-80% of CS cases) of endogenous CS. It is caused by a pituitary adenoma that secretes adrenocorticotropic hormone (ACTH), which stimulates secretion of cortisol by the adrenal glands. If not effectively treated, CD is associated with hypertension, diabetes, obesity, osteoporosis, vascular disease, and shortened life span. Successful resection of a CD-associated ACTH-secreting pituitary adenoma results in immediate biochemical remission and preservation of pituitary function. Early identification of CD by clinical findings, endocrinological evaluation, and imaging studies is critical for diagnosis and effective surgical management. endocrinological and Clinical Features endocrinological Features Normal PhysiologyInsight into the normal and pathophysiological mechanisms of control of the hypothalamic-anterior pituitaryadrenal axis is critical to understanding the pathologic, diagnostic, and therapeutic features of CD (Fig. 1). 52 Under normal physiological conditions (circadian rhythm, where cortisol levels peak in early morning and nadir late at night), systemic inflammation (cytokine), or stress (physiological or psychological), corticotropin-releasing hormone (CRH; a 41-amino acid peptide) is released from the paraventricular hypothalamic nucleus via the median eminence. From the median eminence, CRH is transported in the hypophyseal portal venous system to the pituitary gland, where it binds to the CRH receptor-1 (CRH-R1) on pituitary corticotroph cells. CRH binding to CRH-R1 receptors activates adenylate cyclase and stimulates proopiomelanocortin (POMC) gene expression in the corticotroph cells.17,66 Proopiomelanocortin preprohormone is processed into ACTH (a 39-amino acid peptide) and b-lipotropin (a 93-amino acid peptide). Products of b-lipotropin include b-endorphin and a-lipotropin. ACTH is secreted into the systemic circulation and binds to receptors in the adrenal cortex, where it stimulates production and secretion of glucocorticoids, including cortisol. 83 Under normal conditions, circulating cortisol provides physiological control of this endocrine axis by its negative feedback inhibition on hypothalamic CRH and pituitary ACTH secretion. Cushing's disease (CD) is the result of excess secretion of adrenocorticotropic hormone (ACTH) by a benign monoclonal pituitary adenoma. The excessive secretion of ACTH stimulates secretion of cortisol by the adrenal glands, resulting in supraphysiological levels of circulating cortisol. The pathophysiological levels of cortisol are associated with hypertension, diabetes, obesity, and early death. Successful resection of the CD-associated ACTH-secreting pituitary adenoma is the treatment of choice and results in immediate biochemical remission with preservation of pituitary function. Accurate and early identification of CD is critical for effective surgical management and optimal prognosis. The authors review the cu...

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“…In a another study conducted in 2013,stereotactic radiosurgery was performed in patients with persistent Cushing's disease after prior resection. Tumour control was achieved in 98% patients and new loss of pituitary function occurred in 36% patients [8].…”

Section: Discussionmentioning

confidence: 97%

Hypogonadotropic Hypogonadism with Cushing?s Disease-A Case Report

Jain¹,

Jain²

2015

Clinics Mother Child Health

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A 27 year old woman presented with secondary amenorrhoea. She was obese and had marked pigmentation on face, abdominal striae and severe hypertension. Endocrinal work up revealed low estradiol with low gonadotrophin levels suggesting hypogonadotropic hypogonadism. A magnetic resonance imaging revealed a macroadenoma in the anterior pituitary. A raised cortisol and a raised adenocorticotropic hormone level confirmed the diagnosis of Cushing's disease. The pituitary macroadenoma was resected with Leksell Gamma knife radiosurgery. The response on her reproductive function was immediate, as she had spontaneous menstruation just three weeks after surgery. She also became normotensive and is being kept on follow up.

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Results of Gamma Knife surgery for Cushing's disease

Cited by 89 publications

References 44 publications

The Treatment of Cushing's Disease

The Treatment of Cushing's Disease

Cushing's disease: pathobiology, diagnosis, and management

Hypogonadotropic Hypogonadism with Cushing?s Disease-A Case Report

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