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.
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