Multiple endocrine neoplasia syndromes

Resumen

  • Hereditary tumour syndromes with distinct patterns of organ involvement.
  • Mutations in the MEN1 gene typically cause type 1 multiple endocrine neoplasia (MEN1), and mutations in the RET proto-oncogene typically cause type 2 multiple endocrine neoplasia (MEN2).
  • Prophylactic thyroidectomy in childhood is indicated in MEN2.
  • Medical management of hormonal hypersecretion is important for symptom control.
  • Most tumours require surgical evaluation, although surgical cure is not always possible.
  • Genetic carriers require lifelong monitoring, even after successful operations.
  • Morbidity and mortality result from both hormonal hypersecretion and metastases.
Actualizado por última vez: abr 03, 2013

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