Treatment of sexual precocity by removal of a luteinizing hormone-releasing hormone secreting hamartoma
R. A. Price, P. A. Lee, A. L. Albright, O. K. Ronnekleiv and J. P. Gutai
A 1-year-old infant was initially seen with precocious puberty that
progressed rapidly and was associated with episodic gonadotropin release. A
computed tomographic metrizamide cisternogram showed a pedunculated mass
below the tuber cinereum; the mass was resected. His pubertal development
regressed, his accelerated growth ceased, and his hormone levels returned
to the normal prepubertal range. Immunocytochemical studies of the tissue
demonstrated luteinizing hormone-releasing hormone (LHRH) granules in the
perikaryon of neurons and axons in the hamartoma and axons connecting the
hamartoma to the tuber cinereum. These data suggest that such lesions cause
pubertal development by stimulating gonadotropin secretion via episodic
release of LHRH. If such lesions are pedunculated, operative resection may
be the treatment of choice.
