Hypercortisolaemia due to ectopic adrenocorticotropic hormone secretion by a nasal paraganglioma: a case report and review of the literature.

BACKGROUND: Adrenocorticotropic hormone-producing extraadrenal paragangliomas are extremely rare. We present a case of severe hypercortisolemia due to ectopic adrenocorticotropic hormone secretion by a nasal paraganglioma. CASE PRESENTATION: A 70-year-old Caucasian woman, was emergently admitted to our department with supraventricular tachycardia, oedema of face and extremities and hypertensive crisis. Initial laboratory evaluation revealed severe hypokalemia and hyperglycemia without ketoacidosis, although no diabetes mellitus was previously known. Computed tomography revealed a large tumor obliterating the left paranasal sinus and a left-sided adrenal mass. After cardiovascular stabilisation, a thorough hormonal assessment was performed revealing marked adrenocorticotropic hormone-dependent hypercortisolism. Due to the presence of a cardiac pacemaker magnetic resonance imaging of the hypophysis was not possible. [68Ga-DOTA]-TATE-Positron-Emission-Tomography was performed, showing somatostatin-receptor expression of the paranasal lesion but not of the adrenal lesion or the hypophysis. The paranasal tumor was resected and found to be an adrenocorticotropic hormone-producing paraganglioma of low-proliferative rate. Postoperatively the patient became normokaliaemic, normoglycemic and normotensive without further need for medication. Genetic testing showed no mutation of the succinatdehydrogenase subunit B- and D genes, thus excluding hereditary paragangliosis. CONCLUSION: Detection of the adrenocorticotropic hormone source in Cushing's syndrome can prove extremely challenging, especially when commonly used imaging modalities are unavailable or inconclusive. The present case was further complicated by the simultaneous detection of two tumorous lesions of initially unclear biochemical behaviour. In such cases, novel diagnostic tools - such as somatostatin-receptor imaging - can prove useful in localising hormonally active neuroendocrine tissue. The clinical aspects of the case are discussed and relevant literature is reviewed.

Foods for the prevention of diabetes: how do they work?

With the diabetes epidemic reaching menacing proportions worldwide, there is an urgent need for the development of cost-efficient prevention strategies to be effective at the population level. Great potential in this direction lies in properly designed, large-scale dietary interventions. The macronutrient composition and the caloric content of our diet are major determinants of glucose homeostasis and there is a continuously growing list of foods, nutrients or individual compounds that have been associated with an increased or reduced incidence of diabetes mellitus. These include fat, carbohydrates, fibre, alcohol, polyphenols and other micronutrients or individual dietary compounds, which have been shown to either promote or prevent a progression towards a (pre-)diabetic state. This review aims to briefly summarize relevant epidemiological data linking foods to diabetes and to provide insights into the mechanisms through which these effects are mediated. These include improvement of insulin sensitivity or promotion of insulin resistance, regulation of inflammatory pathways, regulation of glucose transport and tissue glucose uptake, aggravation or attenuation of postprandial glycaemia/insulinaemia, interactions with hormonal responses and ß-cell-dependent mechanisms.

Successful management of severe blunt hepatic trauma by angiographic embolization.

We present the case of an 18-year-old female with severe liver trauma after a motorcycle accident. Due to initial hemodynamic instability, fluid resuscitation and transfusion of two units of red packed cells was required. After stabilization, a CT scan was performed, showing grade V liver injuries according to the American Association for the Surgery of Trauma grading system. Angiography revealed multiple extravasations during the early arterial phase, as well as active extravasation from the proximal left hepatic artery in the late arterial phase. The patient was successfully treated by arterial embolization using metal microcoils, after which no further need for blood transfusion ensued. This report highlights that, in carefully selected cases, arterial embolization can improve the clinical condition of patients, reduce the need for blood transfusion and lessen the possibility of an operation, even if severe liver trauma has ensued.