Résumé
Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.
Sujets)
Femelle , Humains , Hormone corticotrope , Syndrome de Cushing , Tumeurs du médiastin/thérapie , Tumeurs du médiastin/métabolisme , Tumeurs du médiastin , Adulte d'âge moyen , Paragangliome extrasurrénalien/thérapie , Paragangliome extrasurrénalien/métabolisme , Paragangliome extrasurrénalienSujets)
Humains , Mâle , Femelle , Adolescent , Adulte , Adulte d'âge moyen , Tumeurs des bronches/classification , Tumeurs des bronches/complications , Tumeurs des bronches/diagnostic , Tumeurs des bronches/anatomopathologie , Tumeurs des bronches/thérapie , Bronchoscopie , Bronchoscopie/normes , Tumeurs du poumon/classification , Tumeurs du poumon/diagnostic , Tumeurs du poumon/thérapie , Fistule artérioveineuse/diagnostic , Fistule artérioveineuse/étiologie , Fistule artérioveineuse/histoire , Fistule artérioveineuse/thérapie , Paragangliome extrasurrénalien/diagnostic , Paragangliome extrasurrénalien/histoire , Paragangliome extrasurrénalien/anatomopathologie , Paragangliome extrasurrénalien/thérapie , Hamartomes/diagnostic , Hamartomes/thérapieRésumé
Se presentan tres casos de tumores originados en los paraganglios no cromafines localizados en diferentes áreas de la cabeza y el cuello. Se revisan las principales características de estos tumores así como los criterios actuales para su diagnóstico y tratamiento, ya que en el pasado el sistema de paraganglios no cromafines, fue causa de confusión en lo referente a su origen embriológico, su papel fisiológico y la nomenclatura empleada