February 1983

Hamartoma of CNS Associated With Precocious Puberty

Author Affiliations

From the Departments of Pediatrics (Drs Zúñiga, Tanner, and Mosier) and Neurological Surgery (Dr Wild), University of California, Irvine.

Am J Dis Child. 1983;137(2):127-133. doi:10.1001/archpedi.1983.02140280025007

• 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)