Résumé
Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. Carcinoid syndrome significantly and negatively affects patients' quality of life; increases costs compared with the costs of nonfunctioning neuroendocrine tumors; and results in changes in patients' lifestyle, such as diet, work, physical activity and social life. For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed. More than 40 substances have been identified as being potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include serotonin (5-HT), which appears to be the primary marker associated with the syndrome, as well as histamine, kallikrein, prostaglandins, and tachykinins. Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach.
Sujets)
Humains , Cardiopathie carcinoïde/thérapie , Tumeurs neuroendocrines/thérapie , Syndrome carcinoïde malin/thérapie , Imagerie par résonance magnétique , Cardiopathie carcinoïde/physiopathologie , Cardiopathie carcinoïde/imagerie diagnostique , Tumeurs neuroendocrines/physiopathologie , Tumeurs neuroendocrines/imagerie diagnostique , Syndrome carcinoïde malin/physiopathologie , Syndrome carcinoïde malin/imagerie diagnostiqueRésumé
El eritema necrolítico migratorio es una erupción cutánea característica, con hallazgos histopatológicos específicos que se relacionan frecuentemente con un tumor pancreático, el glucagonoma. Los pacientes con este síndrome son generalmente mal diagnosticados. Se presenta un paciente de 54 años de edad con una erupción cutánea recurrente, pérdida de peso, glositis e hiperglucemia de cuatro meses de duración. Esta importante, pero rara enfermedad, puede ser diagnosticada tempranamente, basándonos en el cuadro cutáneo