ABSTRACT
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.
Subject(s)
Female , Humans , Adrenocorticotropic Hormone , Cushing Syndrome , Mediastinal Neoplasms/therapy , Mediastinal Neoplasms/metabolism , Mediastinal Neoplasms , Middle Aged , Paraganglioma, Extra-Adrenal/therapy , Paraganglioma, Extra-Adrenal/metabolism , Paraganglioma, Extra-AdrenalSubject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Bronchial Neoplasms/classification , Bronchial Neoplasms/complications , Bronchial Neoplasms/diagnosis , Bronchial Neoplasms/pathology , Bronchial Neoplasms/therapy , Bronchoscopy , Bronchoscopy/standards , Lung Neoplasms/classification , Lung Neoplasms/diagnosis , Lung Neoplasms/therapy , Arteriovenous Fistula/diagnosis , Arteriovenous Fistula/etiology , Arteriovenous Fistula/history , Arteriovenous Fistula/therapy , Paraganglioma, Extra-Adrenal/diagnosis , Paraganglioma, Extra-Adrenal/history , Paraganglioma, Extra-Adrenal/pathology , Paraganglioma, Extra-Adrenal/therapy , Hamartoma/diagnosis , Hamartoma/therapyABSTRACT
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