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February 1983, Vol 137, No. 2 >
Article | February 1983

Hamartoma of CNS Associated With Precocious Puberty

Oscar F. Zúñiga, MD; Sterling M. Tanner, MD; William O. Wild, MD; H. David Mosier, MD
Am J Dis Child. 1983;137(2):127-133. doi:10.1001/archpedi.1983.02140280025007.
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• A male infant had precocious puberty and hamartoma of the CNS. Signs of puberty appeared and progressed from 6 months of age. A computed tomographic scan disclosed an interpedunculary tumor. A craniotomy was successfully performed at 1½ years of age, and 90% of the tumor was removed. Histologically, the tissue was identified as a hypothalamic hamartoma. Pubertal development stopped. The patient is now 4 years 9 months old and well. Review of medical literature covering a span of 47 years showed 50 cases of hamartomas in or near the hypothalamus confirmed by surgical exploration or autopsy. The male-female ratio of hamartomas with precocious puberty derived from these data is 2:1. Convulsions, mental retardation, or behavioral disorders were present in 48% of the cases; 36% had precocious puberty.

(Am J Dis Child 1983;137:127-133)

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