Multiple endocrine neoplasia: paediatric perspective.
Neuroendocrine tumours constitute a heterogeneous association of neoplasms, originated from a common precursor cell population. They include endocrine glands, such as the pituitary, the parathyroids, the cells of the neuroendocrine adrenals, endocrine islets within glandular tissue (thyroid, pancreas) and dispersed cells (diffuse endocrine system). Neuroendocrine tumours can occur sporadically or in a familial context, such as multiple endocrine neoplasia (MEN) syndromes. These are inherited autosomal dominant cancer syndromes, transmitted with 100% penetrance. They are categorized into MEN type 1 and type 2. The dream of each physician who treats cancer is to develop a strategy that will have a significantly favourable impact on morbidity and mortality associated with malignant tumours. This has been achieved as a result of improved screening and early treatment strategies in MEN. MEN 2 and medullary thyroid carcinoma (MTC) are of special relevance in childhood, because they require urgent and early diagnosis and treatment. The explication of the genetic basis of MTC has revolutionised management of the familial forms of this tumour.
Rodriguez-Sánchez
A
A
Unidad de Metabolismo y Desarrollo, Departamento de Pediatría, Hospital General Universitario Gregorio Marañón, Madrid, Spain. amparorodriguezsanchez@yahoo.es
López-Menchero
C
C
Rodríguez-Arnao
M D
MD
D018276:Carcinoma, Medullary / Q000235:genetics / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005820:Genetic Testing; D006801:Humans; D009377:Multiple Endocrine Neoplasia / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D010673:Pheochromocytoma / Q000235:genetics / Q000473:pathology; D010911:Pituitary Neoplasms / Q000235:genetics / Q000473:pathology; D013964:Thyroid Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery; D013965:Thyroidectomy