Hamartoma of CNS associated with precocious puberty

• Published in: American journal of diseases of children (1960) Vol. 137; no. 2; p. 127
• Main Authors: Zúñiga, O F, Tanner, S M, Wild, W O, Mosier, Jr, H D
• Format: Journal Article
• Language: English
• Published: United States 01.02.1983
• Subjects: Brain Neoplasms - complications; Child; Child Behavior Disorders - complications; Child, Preschool; Diagnosis, Differential; Female; Hamartoma - complications; Hamartoma - diagnosis; Hamartoma - surgery; Humans; Hypothalamic Neoplasms - complications; Hypothalamic Neoplasms - diagnosis; Hypothalamic Neoplasms - surgery; Infant; Infant, Newborn; Intellectual Disability - complications; Male; Puberty, Precocious - diagnosis; Puberty, Precocious - etiology; Seizures - complications; Seizures - diagnosis
• ISSN: 0002-922X
• 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 11/2 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.