Diagnostic workup of Cushing’s syndrome

Transsphenoidal surgery is the first-line treatment for Cushing's disease to selectively remove the tumor. The rate of postoperative remission is estimated around 70%-80% in expert centers. However, the long-term remission rate is lower because of recurrence during follow-up that can be observed in 15% to 25% of the patients depending on the studies and duration of follow-up. There is no significant predictive factor of recurrence before surgery, but postoperative corticotroph insufficiency and its duration has been found to be a protective factor for recurrence in many studies.The persistence of a positive response to desmopressin after surgery is associated with a higher rate of recurrence. Long term monitoring for recurrence with annual clinical and hormonal investigations after the hypothalamic-pituitary-adrenal axis postoperative recovery is advised. The biological tests used for the diagnosis of Cushing's syndrome (24 h-urinary-free cortisol [UFC], late-night salivary or serum cortisol, 1 mg dexamethasone suppression test) can be used to screen for recurrence.Several studies report that increased late night cortisol and alterations of dynamic testing can be observed before the increased 24 h-UFC. For this reason it is suggested that late-night salivary cortisol would be a very sensitive tool to diagnose recurrence, pending the realization of several assays in case of borderline or discrepant result. This review will summarize the knowledge about recurrence of Cushing's disease after pituitary surgery and the current recommendations for its monitoring and diagnosis.

Section: Discussionmentioning

confidence: 99%

Long‐term follow‐up and predictors of recurrence of Cushing's disease

Guignat

Bertherat

2022

“…This article is part of an update series on the diagnosis and treatment of Cushing's syndrome 90,109,113–127 …”

Section: Discussionmentioning

confidence: 99%

The diagnosis and management of Cushing's syndrome in pregnancy

Hamblin

Coulden

Fountas

et al. 2022

Endogenous Cushing's syndrome (CS) is rarely encountered during pregnancy. Clinical and biochemical changes in healthy pregnancy overlap with those seen in pregnancy complicated by CS; the diagnosis is therefore challenging and can be delayed. During normal gestation, adrenocorticotrophic hormone, corticotrophin‐releasing hormone, cortisol, and urinary free cortisol levels rise. Dexamethasone administration fails to fully suppress cortisol in pregnant women without CS. Localisation may be hindered by non‐suppressed adrenocorticotrophic hormone levels in a large proportion of those with adrenal CS; smaller corticotroph adenomas may go undetected as a result of a lack of contrast administration or the presence of pituitary hyperplasia; and inferior petrosal sinus sampling is not recommended given the risk of radiation and thrombosis. Yet, diagnosis is essential; active disease is associated with multiple insults to both maternal and foetal health, and those cured may normalise the risk of maternal–foetal complications. The published literature consists mostly of case reports or small case series affected by publication bias, heterogeneous definitions of maternal or foetal outcomes or lack of detail on severity of hypercortisolism. Consequently, conclusive recommendations, or a standardised management approach for all, cannot be made. Management is highly individualised: the decision for surgery, medical control of hypercortisolism or adoption of a conservative approach is dependent on the timing of diagnosis (respective to stage of gestation), the ability to localise the tumour, severity of CS, pre‐existing maternal comorbidity, and, ultimately, patient choice. Close communication is a necessity with the patient placed at the centre of all decisions, with risks, benefits, and uncertainties around any investigation and management carefully discussed. Care should be delivered by an experienced, multidisciplinary team, with the resources and expertise available to manage such a rare and challenging condition during pregnancy.

“…This article is part of an update series on the diagnosis and treatment of Cushing's syndrome. [27][28][29][30][31][32][33][34][35][36][37][38][39][40][41][42]…”

Section: Discussionmentioning

confidence: 99%

Role of radiosurgery in the treatment of Cushing's disease

Losa

Albano

Bailo

et al. 2022

Radiotherapy is a useful adjuvant treatment for patients with Cushing's disease that is not cured by surgery. In particular, Gamma Knife radiosurgery (GKRS) has been increasingly used worldwide as the preferred radiation technique in patients with persistent or recurrent Cushing's disease. The most widely accepted criterion for hormonal remission after GKRS is normalization of urinary free cortisol (UFC) levels.