A neonate is born with a hypothalamic hamartoma and presents with precocious puberty at age 2 years. Laboratory studies show elevated LH and FSH with pubertal response to GnRH stimulation. Which of the following best explains the mechanism by which hypothalamic hamartomas cause central precocious puberty?
- A Ectopic GnRH secretion from hamartoma neurons ✓
- B Autonomous estrogen production by the hamaroma
- C Premature activation of ovarian GnRH receptors
- D Destruction of the arcuate nucleus feedback inhibition
Correct answer: A. Ectopic GnRH secretion from hamartoma neurons
Explanation
Hypothalamic hamartomas causing precocious puberty contain ectopic GnRH-secreting neurons that function as an accessory pulse generator, secreting GnRH in a pulsatile pattern independent of normal hypothalamic regulation. This drives pituitary gonadotropin secretion prematurely. It is NOT due to sex steroid production or direct gonadal activation, as evidenced by the pubertal GnRH stimulation response.
Reference: Williams Textbook of Endocrinology, 14th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.