A review of Cushing's disease treatment by the Department of Neuroendocrinology of the Brazilian Society of Endocrinology and Metabolism

ABSTRACT The treatment objectives for a patient with Cushing's disease (CD) are remission of hypercortisolism, adequate management of co-morbidities, restoration of the hypothalamic-pituitary-adrenal axis, preservation of fertility and pituitary function, and improvement of visual defects in cases of macroadenomas with suprasellar extension. Transsphenoidal pituitary surgery is the main treatment option for the majority of cases, even in macroadenomas with low probability of remission. In cases of surgical failure, another subsequent pituitary surgery might be indicated in cases with persistent tumor imaging at post surgical magnetic resonance imaging (MRI) and/or pathology analysis of adrenocorticotropic hormone-positive (ACTH+) positive pituitary adenoma in the first procedure. Medical treatment, radiotherapy and adrenalectomy are the other options when transsphenoidal pituitary surgery fails. There are several options of medical treatment, although cabergoline and ketoconazole are the most commonly used alone or in combination. Novel treatments are also addressed in this review. Different therapeutic approaches are frequently needed on an individual basis, both before and, particularly, after surgery, and they should be individualized. The objective of the present review is to provide the necessary information to achieve a more effective treatment for CD. It is recommended that patients with CD be followed at tertiary care centers with experience in treating this condition.

Hipercortisolismo subclínico / Subclinical hypercortisolism

El hipercortisolismo subclínico se define como una alteración del eje hipotálamo hipófisis adrenal en ausencia de los síntomas y signos clásicos del exceso de cortisol. Es una enfermedad endocrina que se presenta hasta en 20 por ciento de los pacientes a los cuales se les diagnostica un tumor adrenal. Las investigaciones realizadas, sugieren la hipótesis que puede estar asociado a complicaciones crónicas como la hipertensión arterial, la diabetes mellitus, el sobrepeso/obesidad y la osteoporosis. El diagnóstico y tratamiento del hipercortisolismo subclínico se han convertido actualmente en un tema de creciente interés y debate, pero aún no existe consenso. Se necesitan estudios prospectivos y aleatorizados para confirmar la hipótesis propuesta, establecer la mejor forma de realizar el diagnóstico y de seleccionar los pacientes que se beneficien con el tratamiento quirúrgico(AU)

Subclinical hypercortisolism is an alteration of the hypothalamus-hypophysis-adrenal axis in absence of classical symptoms and signs of excessive cortisol. It is an endocrine disease that affects up to 20 percent of patients diagnosed with an adrenal tumor. The research studies indicate the hypothesis that this entity could be associated to chronic complications such as blood hypertension, diabetes mellitus, overweight/obesity and osteoporosis. The diagnosis and treatment of subclinical hypercortisolism has turned into a topic of growing interest and discussion nowadays, but the consensus is still pending. It is then necessary to perform prospective and randomized studies in order to confirm the suggested hypothesis, to determine the best way of making a diagnosis and to select the patients who are going to really benefit from a surgical treatment(AU)

Pediatric Cushing's syndrome: clinical features, diagnosis, and treatment: [review]

Cushing's syndrome (CS) results from prolonged exposure to supraphysiological levels of circulating glucocorticoids, endogenously or exogenously derived. Although rare in childhood, CS remains a difficult condition to diagnose and treat. A multidisciplinary approach and close collaboration with adult colleagues is adopted at most large centres that manage pediatric CS patients. Although pediatric protocols are derived from adult data, significant differences exist between adult and childhood CS. Furthermore, long term outcome parameters including final height, bone mineral density, reproductive function, body composition and psychological health pose challenges for pediatric care. This article will aim to provide an overall view of pediatric CS highlighting some of the differences between adult and pediatric CS.

A síndrome de Cushing (SC) resulta da exposição prolongada a níveis suprafisiológicos de glicocorticóides circulantes, tanto endógenos como de seus derivados exógenos. Embora rara na infância, a SC permanece uma condição difícil de ser diagnosticada e tratada. Uma avaliação multidisciplinar e a colaboração próxima com colegas da área não-pediátrica são adotadas na maioria dos grandes centros que cuidam de pacientes pediátricos com SC. Embora os protocolos pediátricos sejam derivados de dados em adultos, existem diferenças significativas entre a SC no adulto e na infância. Além disso, parâmetros evolutivos finais, incluindo altura final, densidade mineral óssea, função reprodutiva, composição corporal e saúde psicológica trazem desafios no cuidado pediátrico. Este artigo procura oferecer uma visão geral da SC pediátrica, focalizando algumas das diferenças entre a SC adulta e a pediátrica.