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La sindrome di Nelson-Salassa

  • Roberto Toni,
  • Fulvio Barbaro,
  • Giusy Di Conza,
  • Salvatore Mosca,
  • Ronald M. Lechan

摘要

Nelson’s syndrome, also called post-adrenalectomy syndrome, was first described in 1958 by Don H. Nelson, who identified a clinical triad of cutaneous hyperpigmentation, visual defects, and secretion of ACTH from a pituitary tumour. Although it is now clear that the triad occurs in only a minority of patients, evidence that reduced/abolished corticosteroid feedback acted as a stimulus to the growth/progression of an ACTH-secreting pituitary tumour confirms the experimental data presented by pathologist Jacob Furth. He first proposed that pituitary tumours could develop as a result of altered inhibitory feedback by peripheral hormones, and suggested that MSH could be co-secreted with ACTH, a concept unknown at that time. In 1959, Mayo Clinic endocrinologist Robert M. Salassa described seven similar cases and observed these tumours could be aggressive, locally invasive, and give rise to distant metastases; therefore, the syndrome is now referred to as Nelson-Salassa syndrome.