Cyclic Cushing's Syndrome: An Overview

Mantero, Franco; Scaroni, Carla; Albiger, Nora

doi:10.1007/s11102-005-4025-5

Cited by 31 publications

(17 citation statements)

References 65 publications

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“…This would imply that regular cyclicity with '12 h to 85 days' periodicity, as has been previously suggested, can clearly occur, but seems much less frequent than more irregular and unpredictable fluctuations. (2,10,25). Isolated clinical and biochemical cycles showed similar frequencies: clinical along with biochemical cycles were the most commonly identified, implying no additive information from laboratory data for the investigation of cyclicity.…”

Section: Discussionmentioning

confidence: 99%

“…The molecular basis for this significant variability in hormonal release has been little studied in molecular terms (36). Several hypotheses have been suggested to explain the phenomenon, including episodic haemorrhage, the synchronous growth and death of tumour cells (9,10) or fluctuations in adrenal-pituitary axis feedback (14). It seems unlikely that there is abnormal hypothalamic control as such regulation should be absent in the presence of an autonomous tumour (9,28,(37)(38)(39)(40).…”

Section: Discussionmentioning

confidence: 99%

“…It has been more frequently described in pituitary-dependent CS, Cushing's disease, having being characterized by periodic increases and decreases in cortisol levels, clinically and/or biochemically documented (2,7,8). However, it has been considered to be an uncommon phenomenon, since only a few small series and case reports confirm its presence (9,10). It is important to consider that acute investigation may be associated with episodes of lowered activity and delays in definitive diagnosis.…”

Section: Introductionmentioning

confidence: 99%

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The prevalence and characteristic features of cyclicity and variability in Cushing's disease

Alexandraki¹,

Kaltsas²,

Isidori

et al. 2009

European Journal of Endocrinology

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Objective: Cyclical Cushing's syndrome may render the diagnosis and management of Cushing's disease difficult. The aim of the present study was to investigate the prevalence of cyclicity and variability in patients with Cushing's disease, and to identify putative distinctive features. Design: Retrospective case-note study. Methods: We analysed the case records of 201 patients with Cushing's disease in a retrospective casenote study. Cyclicity was considered as the presence of at least one cycle, defined as a clinical and/or biochemical hypercortisolaemic peak followed by clinical and biochemical remission, followed by a new clinical and/or biochemical hypercortisolaemic peak. The fluctuations of mean serum cortisol levels, as assessed by a 5-point cortisol day curve, defined the variability. Results: Thirty (14.9%; 26 females) patients had evidence of cyclicity/variability. 'Cycling' patients were older but no difference in sex or paediatric distribution was revealed between 'cycling' and 'noncycling' patients. The median number of cycles was two for each patient, and 4 years was the median intercyclic period. A trend to lower cure rate post-neurosurgery and lower adenoma identification was observed in 'cycling' compared with 'non-cycling' patients. In multivariate analysis, older patients, longer follow-up, female sex and no histological identification of the adenoma were associated with an increased risk of cyclic disease. Conclusions: This large population study reveals that cyclicity/variability is not an infrequent phenomenon in patients with Cushing's disease, with a minimum prevalence of 15%. Physicians should be alert since it can lead to frequent problems in diagnosis and management, and no specific features can be used as markers.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The prevalence and characteristic features of cyclicity and variability in Cushing's disease

Alexandraki¹,

Kaltsas²,

Isidori

et al. 2009

European Journal of Endocrinology

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“…It has been proposed that episodic haemorrhage or synchronic growth and death of ACTH-or cortisol-producing tumour cells may lead to periodic hypercortisolism (67). In support of this theory, necrosis has been found in removed corticotroph …”

Section: Pathophysiology Of Cyclic Csmentioning

confidence: 98%

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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“…8 The pathophysiology has yet to be defined clearly, although some theories have been proposed. A histological find ing of necrosis within tumors led some investigators to propose that episodic hemorrhage and necrosis of corti solproducing cells causes a cyclical release of cortisol, 7 although others have identified cases of cyclical CS with out necrosis in the tumor specimen. 2 Another theory pos tulates that tumor calcifications lead to infarction with subsequent eucortisolemia; 4 however, this theory does not explain the periodicity.…”

Section: Discussionmentioning

confidence: 99%

Cyclic Cushing's disease with misleading inferior petrosal sinus sampling results during a trough phase

Bonert

Bose

Carmichael

2015

FOC

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Diagnosing Cushing's syndrome is challenging and is further hampered when investigations are performed in a patient with cyclic Cushing's syndrome. A subset of patients with Cushing's syndrome exhibit periods of abnormal cortisol secretion with interspersed normal secretion. Patients can have periods of clinical improvement during these quiescent phases or remain symptomatic. Initial diagnostic testing can be challenging because of the unpredictable durations of the peak and trough phases, and it is especially challenging when the diagnosis of cyclic Cushing's syndrome has not yet been determined. Here, the authors present the case of a patient with Cushing's disease with a pathology-proven adrenocorticotropic hormone (ACTH)–secreting pituitary adenoma and whose initial inferior petrosal sinus sampling (IPSS) results were deemed indeterminate; further studies elucidated the diagnosis of cyclic Cushing's syndrome. Repeat IPSS was diagnostic of a central source for ACTH secretion, and the patient was treated successfully with transsphenoidal resection. Literature concerning the diagnosis and management of cyclic Cushing's syndrome is also reviewed.

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Cyclic Cushing's Syndrome: An Overview

Cited by 31 publications

References 65 publications

The prevalence and characteristic features of cyclicity and variability in Cushing's disease

The prevalence and characteristic features of cyclicity and variability in Cushing's disease

Cyclic Cushing’s syndrome: a clinical challenge

Cyclic Cushing's disease with misleading inferior petrosal sinus sampling results during a trough phase

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