The Long-Term Outcome of Pituitary Irradiation after Unsuccessful Transsphenoidal Surgery in Cushing's Disease

Estrada, Javier; Boronat, Mauro; Mielgo, Mercedes; Magallón, Rosa; Millán, Isabel; Diez, Santiago; Lucas, T.; Barceló, B

doi:10.1056/nejm199701163360303

Cited by 359 publications

(168 citation statements)

References 28 publications

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“…The risk of CD recurrence can reach 25%, 3 years after surgery (Patil et al 2008). Second and third line therapies such as a second pituitary surgery (Patil et al 2008, Fleseriu 2012, pituitary irradiation (conventional and/or stereotactic) (Estrada et al 1997, Tritos et al 2011 and bilateral adrenalectomy (Young & Thompson 2005, Assie et al 2007, Chow et al 2008, Ritzel et al 2013) have been utilized with variable results and specific complications.…”

Section: Overview Of CD Therapymentioning

confidence: 99%

Treatment of Cushing's disease: a mechanistic update

Cuevas‐Ramos¹,

Fleseriu²

2014

Journal of Endocrinology

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Cushing's disease (CD) is characterized by an ACTH-producing anterior corticotrope pituitary adenoma. If hypothalamus-pituitary-adrenal (HPA) axis physiology is disrupted, ACTH secretion increases, which in turn stimulates adrenocortical steroidogenesis and cortisol production. Medical treatment plays an important role for patients with persistent disease after surgery, for those in whom surgery is not feasible, or while awaiting effects of radiation. Multiple drugs, with different mechanisms of action and variable efficacy and tolerability for controlling the deleterious effects of chronic glucocorticoid excess, are available. The molecular basis and clinical data for centrally acting drugs, adrenal steroidogenesis inhibitors, and glucocorticoid receptor antagonists are reviewed, as are potential novel molecules and future possible targets for CD treatment. Although progress has been made in the understanding of specific corticotrope adenoma receptor physiology and recent clinical studies have detected improved effects with a combined medical therapy approach, there is a clear need for a more efficacious and better-tolerated medical therapy for patients with CD. A better understanding of the molecular mechanisms in CD and of HPA axis physiology should advance the development of new drugs in the future.

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Section: Overview Of CD Therapymentioning

confidence: 99%

Treatment of Cushing's disease: a mechanistic update

Cuevas‐Ramos¹,

Fleseriu²

2014

Journal of Endocrinology

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“…Thus, neurosurgical treatment, if possible using minimally invasive endoscopic techniques, is the primary therapy for Cushing's disease (16). Pituitary irradiation, which may comprise conventional irradiation or newer techniques employing stereotactic radiosurgery with the g-knife or the linear accelerator, is a second treatment option (105). In exceptional cases, bilateral adrenalectomy may be an additional treatment option.…”

Section: Treatmentmentioning

confidence: 99%

Cyclic Cushing’s syndrome: a clinical challenge

Meinardi¹,

Wolffenbuttel²,

Dullaart³

2007

eur j endocrinol

125

134

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Cyclic Cushing's syndrome (CS) is a rare disorder, characterized by repeated episodes of cortisol excess interspersed by periods of normal cortisol secretion. The so-called cycles of hypercortisolism can occur regularly or irregularly with intercyclic phases ranging from days to years. To formally diagnose cyclic CS, three peaks and two troughs of cortisol production should be demonstrated. Our review of 65 reported cases demonstrates that cyclic CS originates in 54% of cases from a pituitary corticotroph adenoma, in 26% from an ectopic ACTH-producing tumour and in about 11% from an adrenal tumour, the remainder being unclassified. The pathophysiology of cyclic CS is largely unknown. The majority of patients with cyclic CS have clinical signs of CS, which can be either fluctuating or permanent. In a minority of patients, clinical signs of CS are absent. The fluctuating clinical picture and discrepant biochemical findings make cyclic CS extremely hard to diagnose. Clinicians should therefore be aware of this clinical entity and actively search for it in all patients with suspected CS but normal biochemistry or vice versa. Frequent measurements of urinary cortisol or salivary cortisol levels are a reliable and convenient screening tool for suspected cyclic CS. Cortisol stimulation or suppression tests may give spurious results owing to spontaneous falls or rises in serum cortisol at the time of testing. When cyclic CS is biochemically confirmed, further imaging and laboratory studies are guided by the presence or absence of ACTH dependency. In cases of suspected ectopic ACTH production, specific biochemical testing for carcinoids or neuroendocrine tumours is required, including measurements of serotonin in platelets and/or urine, chromogranin A and calcitonin.European Journal of Endocrinology 157 245-254

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“…Experienced surgeons achieve an initial biochemical remission in 70-80% of cases, but a significant number of recurrences are seen during long-term follow-up, the rate ranging between 9% and 23% (48,49). Pituitary radiation is reserved for patients failing to respond to surgery, but this treatment may only be effective after several years and it causes a high rate of pituitary insufficiency (50).…”

Section: Therapymentioning

confidence: 99%