Key words: Central precocious puberty, Hypothalamic hamartoma, Case report
The paper describes a case of a child with rapidly progressive central puberty (PPC) secondary
to hypothalamic hamartoma. Hypothalamic hamartoma is a congenital malformation
diagnosed with MRI. It may be associated with PPC, gelastic seizures and moderate
mental retardation. Precocious puberty is defined by the appearance of pubertycompatible
symptoms at an age of less than 2.5 SD from the mean, before the age of
eight in girls and nine in boys. The prevalence is 10 times higher in girls. The organic
forms recognise as a mechanism the premature activation of gonadotropin-releasing hormone
secretion, secondary to tumours or hypothalamic lesions. Idiopathic forms are
more common in females and occur in the 6-8-age group. In males the organic forms
cover about 50% of the possibilities and therefore there is always the indication to perform
an MRI. Often no therapy is needed especially when the onset is in the 6-8-age
range in females, and around the age of 9 in males. In cases with progressive PPC,
there may be unfavourable psychosocial problems and short stature, therefore the indication
to therapy will have to be discussed with patients’ families case by case.
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