GYN TRIAD
Granulosa Cell Ovarian Tumor
Precocious complete isosexual puberty
6-year-old girl
Pelvic mass
Gonadotropin-dependent occurs because of increased secretion of estrogens
that are dependent on premature release of gonadotropins from the
hypothalamus and pituitary.
Idiopathic (80% of cases): The most common explanation is
constitutional without a pathologic process present. Patient typically age 6–
- The diagnosis is usually one of exclusion after CNS imaging is shown to
be normal. Management: GnRH agonist suppression (leuprolide or
Lupron) of gonadotropins until appropriate maturity or height has been
reached.
CNS pathology (rare): A CNS pathologic process stimulates
hypothalamic release of GnRH, which leads to FSH release and ovarian
follicle stimulation of estrogen production. This may include
hydrocephalus, von Recklinghausen disease, meningitis, sarcoid, and
encephalitis. CNS imaging is abnormal. Patient typically age <6.
Management: Directed at the specific pathologic process.
Gonadotropin-independent occurs when estrogen production is independent
of gonadotropin secretion from the hypothalamus and pituitary.
McCune-Albright syndrome (or polyostotic fibrous dysplasia) (5% of
cases) is characterized by autonomous stimulation of aromatase enzyme
production of estrogen by the ovaries. The syndrome includes multiple
cystic bone lesions and café au lait skin spots. Management: Aromatase