Chapter 11a: Pituitary and hypothalamic tumour syndromes in childhood

Abstract

Central nervous system (CNS) tumours are the second commonest childhood malignancy. and survial has increased as a result of improved multimodality cancer therapies and better supportive care. Measurements of PRL, AFP and β-hCG are essential prior to commencement of any therapy. Craniopharyngiomas and low grade gliomas account for most tumors, while pituitary adenomas are rare. Non-neoplastic masses include pituitary hyperplasia and Rathke’s cleft cysts. Neurological syndromes and endocrine dysfunction must be recognized both before treatment and after. Both the original tumor and its treatment may disturb GH secretion, cause gonadotophin deficiency, or Posterior Pituitary Dysfunction, and less commonly reduce thyroid or adrenal function. The “hypothalamic syndrome” including variable hypothalamic dysfunction and morbid obesity is a common sequelae of surgical treatment, presumably caused by dysregulation of anorexigenic and orexigenic hormone signals

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