Abstract
A hypothalamic hamartoma associated with true precocious puberty in a 2-year-old girl is reported. Following subtotal removal of the tumor, endocrinological abnormalities as well as clinical manifestations of precocious puberty were restored to normal. Although the immunofluorescence study of the specimen was negative, ultrastructural studies revealed dense core vesicles of approximately 100 nm in size in the neurons. The mechanisms of precocious puberty in patients with a hypothalamic hamartoma are briefly discussed.