[Precocious puberty, apparently idiopathic, in a 4-year-old boy].
Explore the source record for details and available documents.
SEARCH · PubMed Health
Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Tumors are rare, but well-documented causes of precocious puberty in both sexes. The therapeutic and prognostic implications of a diagnosis of cancer require that the presence of a neoplastic process be ruled out in any case of precocious puberty. Granulosa-cell tumor of the ovary and Leydig-cell tumor of the testis are the most frequent gonadal tumors inducing precocious pseudopuberty in the two sexes. Adrenal tumors sustain a variety of endocrine syndromes, the most frequent one being virilization with or without hypercortisolism. Pure feminizing adrenal neoplasms have been described. For reasons not yet well understood, hypothalamochiasmatic glioma (beta-HCG) secreting tumors have almost never been described in association with female precocious puberty. Among these neoplasia, pineal germ-cell tumor inducing sexual maturation must be included. Hypothalamochiasmatic glioma and craniopharyngioma are the two cerebral tumors capable of inducing true precocious puberty. Even if equally distributed between both sexes, these tumors interfere with sexual maturation less frequently in girls than in boys. Hypothalamic hamartoma is considered a benign tumor, since it does represent a space-occupying mass. It more correctly could be called a malformation if its histologic characteristics are recalled. This cerebral lesion is now frequently described in children with true precocious puberty, probably because of improved diagnostic imaging methods.
Growth hormone when used in precocious puberty in combination with Gonadotropin releasing hormone analogue (GnRHa) instead of using GnRHa alone has been shown to improve final height prognosis. We report here a two-year follow-up of three cases of precocious puberty, two of whom were treated with a combination of GH and GnRHa and the third treated with GnRHa alone.
The syndrome of isosexual precocious puberty (PP) associated with a primary malignant hepatic tumor is rare and previously reported in only 17 cases with poor prognosis. Twelve cases are well-documented gonadotropin-producing tumors. We here describe a new case of virilizing hepatoblastoma in a 2-year-10-month-old boy with evidence of testosterone (T) production by the tumor itself, and survival with a 3 1/2-year follow-up after an extended right hepatic lobectomy. Preoperative laboratory findings showed high levels of serum alpha-fetoprotein (AFP) and T:350,000 ng/mL and 4.92 ng/mL, respectively, which normalized after surgery. There was no circulating gonadotropin nor stimulation of the hypothalamic-pituitary axis. Testicular biopsy showed neither interstitial-cell maturation nor Leydig-cell hyperplasia. Moreover, demonstration of T secretion by tumor cells and T synthesis in presence of C14 progesterone was performed in an in vitro culture system. These data seem to provide supportive evidence of a T-producing hepatoblastoma.
The problems of central precocious puberty (CPP) are serious enough to the patient to deserve treatment. There is a general consensus among paediatric endocrinologists that the treatment of true CPP (i.e., in children young enough to have a formal diagnosis) is indicated in many cases. In children with modestly early puberty who are not fulfilling the diagnostic criteria, this is not the case. The treatment of choice is a gonadotropin-releasing hormone (GnRH) analogue. Prolonged analogues are more effective than short-acting ones and, most importantly, independent of patient compliance. Data on agonists have accumulated over two decades and evidence of effects is rich in girls but sparse in boys. GnRH agonists are generally effective and safe drugs; the suppression of puberty is reversible and there is much information on GnRH agonists for the treatment of CPP showing very few adverse effects and the effects on final height are well documented in girls < 6 years of age. There is some (but not highly convincing) evidence for their effect on final height for those of 6 - 8 years of age and there is no evidence for an increase in final height after the age of 8 years in girls. If a decision to have treatment is taken, treatment should start immediately as a possible benefit is less probable if the start of treatment is delayed. When treatment should be stopped is a matter of controversy. Combination with growth hormone increases final height, but the clinical relevance can be discussed as well as the health economy aspects. The limits of indications are still to be defined.