Metyrapone is effective therapy for short- and long-term control of hypercortisolemia in CS.

Background: The reviewing and assessment of epidemiological characteristics of neuroendocrine tumours (NETs) remains a challenge. Despite the fact that it is an uncommon family of neoplasms, several worldwide series have revealed an increasing incidence of this rare condition. However, the data are difficult to compare over time due to changes in classification. Methods: We compared the data related to incidence, prevalence, stage of the disease at diagnosis and survival reported in several series, focusing on the differences and trying to examine some of the probable reasons that may explain the variations in the results between studies. Results and Conclusions: The incidence of NETs is increasing over time, and their incidental discovery due to improved and more frequent imaging does not seem to be enough to explain this rise. Significant differences can be found between geographic regions and races, suggesting that environmental or genetic factors may contribute to the clinical and biological behaviour of these tumours; increasing our knowledge of oncogenesis will be necessary to explain them. As with other rare diseases, creating specific databases and multidisciplinary working groups would improve the accuracy of the information gained.

Objective: Flushing can be defined as a sensation of warmth accompanied by erythema that most commonly is seen on the face and which occurs in episodic attacks. Such a problem can be clinically problematic, since many conditions and drugs can be related to flushing, and while often there appears to be no underlying organic disease, it is important to exclude disorders since they may be life-threatening conditions. Design and methods: We performed a search in MEDLINE using the terms 'flushing' in combination with 'carcinoid syndrome', 'pheochromocytoma', 'mastocytosis', 'menopausal hot flush' and 'treatment'. European and American guidelines relating to neuroendocrine tumours, mastocytosis and menopause were reviewed. Results: In this review, we discuss the main causes of flushing and propose an algorithm based on pathogenesis, which can be used to guide the clinical evaluation process. We also review recent significant developments in the assessment and treatment of the carcinoid syndrome and menopausal hot flushes, which should guide the clinical practice regarding this common but sometimes confusing condition. Conclusions: When evaluating flushing, a precise systematic approach is needed to exclude potentially serious underlying causes, although despite this, the cause of the disorder is not always found. If symptoms are not progressive, the patient should be advised about its apparently benign nature in order to avoid unnecessary studies or initiating treatments of minimal benefit.