Human Chorionic Gonadotropin-Secreting Pineal Tumors Relation to Pathogenesis and Sex Limitation of Sexual Precocity*

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Abstract
We report a 5 6/12-yr-old male who developed incomplete sexual precocity due to a hCG-secreting tumor in the region of the pineal gland. The patient presented with evidence of increased intracranial pressure (Parinaud's sign and papilledema) and was noted to have enlarged testes (right, 3.5 × 2.5 cm; left, 3.0 × 2.5 cm). Initial hormonal studies showed an adult male level of testosterone (600 ng/dl) and high plasma concentrations of immunoreactive LH, which proved to be hCG, not LH, when assayed in a RIA specific for hCG. The iv administration of LRF did not elicit a rise in plasma LH values. A mass in the region of the pineal gland was documented by pneumoencephalogram and cerebral arteriogram. The patient was treated with 5000 rads to the cranium. Two years postirradiation (age, 7 10/12 yr) there was complete involution of the tumor, undetect ;ble plasma levels of hCG and regression of pubertal development associated with prepubertal plasma concentrations of testosterone and a prepubertal LH response to LRF. Laboratory evidence of partial and later severe GH deficiency was present. At age 9 6/12 yr, the patient developed physical and hormonal evidence of true central puberty. Six years postirradiation, there was no evidence of tumor recurrence by computed tomography brain scan, and plasma hCG has remained undetectable. The present case illustrates the development of sexual precocity independent of the activation of the hypothalamic-pituitary-gonadotropin axis in a patient with an hCG-secreting tumor in the pineal region, followed by regression of the tumor and the sexual precocity after irradiation and later by the onset of normal puberty. We suggest that many pineal tumors cause incomplete rather than true precocious puberty via the production of hCG; this would explain in part the limitation of sexual precocity to males.