中枢神经系统错构瘤伴性早熟。

Hamartoma of CNS associated with precocious puberty.

作者信息

Zúñiga O F, Tanner S M, Wild W O, Mosier H D

出版信息

Am J Dis Child. 1983 Feb;137(2):127-33. doi: 10.1001/archpedi.1983.02140280025007.

DOI:10.1001/archpedi.1983.02140280025007

Abstract

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.

摘要

一名男婴患有中枢神经系统错构瘤和性早熟。青春期体征从6个月大时开始出现并进展。计算机断层扫描显示脚间肿瘤。患儿在1岁半时成功接受了开颅手术，90%的肿瘤被切除。组织学检查显示该组织为下丘脑错构瘤。青春期发育停止。患儿现4岁9个月，情况良好。回顾47年的医学文献发现，经手术探查或尸检证实有50例下丘脑或其附近错构瘤病例。根据这些数据，患有性早熟的错构瘤的男女比例为2:1。48%的病例出现惊厥、智力迟钝或行为障碍；36%的病例患有性早熟。