Which clinical history findings are characteristic of precocious puberty in McCune-Albright syndrome (MAS)?

Updated: Jan 17, 2019
  • Author: Gabriel I Uwaifo, MD; Chief Editor: George T Griffing, MD  more...
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Answer

Precocious puberty can result from either central gonadotropin-dependent causes or peripheral gonadotropin-independent causes (see Etiology). [5] Symptoms of an intracranial process (eg, abrupt vision changes, nighttime headaches, or nighttime emesis) are suggestive of hypothalamic lesions that can lead to gonadotropin-dependent precocious puberty and are not consistent with MAS. Previous brain injury due to infection or trauma is also associated more often with central precocious puberty.

Although the following pattern is not universal, the [peripheral gonadotropin-independent puberty in MAS tends to be seen more frequently with vaginal bleeding or breast development unaccompanied by growth of pubic hair and tends to occur at an earlier age than central gonadotropin-dependent precocious puberty. Furthermore, vaginal bleeding often occurs before the onset of breast development and tends to be irregular. Bleeding episodes may be isolated or frequently recurrent, with very little pattern or predictability.

In patients with precocious vaginal bleeding or breast development, other possible causes of estrogen excess must be considered. Accidental ingestion of estrogen supplements can cause breast development, increased height velocity, and maturation of the endometrial lining. As estrogen levels decrease, withdrawal bleeding can occur. If vaginal bleeding occurs in the absence of other signs of estrogen excess (eg, breast development or increased height velocity), a careful history mindful of possible trauma or sexual abuse should be obtained.

Forms of sexual precocity are observed in more than 50% of women with MAS. [29] Sexual precocity also occurs in male patients but is less common. Some MAS patients may have normal onset of puberty at a normal age.


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