Hamartoma of CNS associated with precocious puberty.
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.
D001932:Brain Neoplasms / Q000150:complications*; D002648:Child; D002653:Child Behavior Disorders / Q000150:complications; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006222:Hamartoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007029:Hypothalamic Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000150:complications; D008297:Male; D011629:Puberty, Precocious / Q000175:diagnosis / Q000209:etiology*; D012640:Seizures / Q000150:complications / Q000175:diagnosis