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[一例因6年生长发育迟缓后发现鞍上区黄色肉芽肿的儿科病例]

[A Pediatric Case of Xanthogranuloma in the Suprasellar Region Detected by a Severe Short Stature after 6 Years Growth Failure].

作者信息

Shimamoto Taro, Yamamoto Yukiyo, Kuwamura Mami, Asai Hiroshi, Ishii Masahiro, Honda Yuko, Kusuhara Koichi

机构信息

Department of Pediatrics, School of Medicine, University of Occupational and Environmental Health, Japan.

Director of Medical Education, School of Medicine, University of Occupational and Environmental Health, Japan.

出版信息

J UOEH. 2019;41(2):249-257. doi: 10.7888/juoeh.41.249.

Abstract

Here we report a case of a 12-year-old girl who was referred to our department because of marked short stature of more than -5 SD below the median. Although her growth failure began suddenly at 6 years of age, she never had an examination because she had no other symptoms. Brain MRI examination suggested a tumor in the suprasellar region, and endocrine examination revealed combined pituitery hormone deficiency due to the tumor. Before surgery, the supplementation with hydrocortisone and levothyroxine was initiated. The pathological diagnosis of the surgically removed tumor was xanthogranuloma. The pattern of her growth curve showed a growth failure with sudden onset, which is a typical pattern of short stature secondary to pituitary disfunction including growth hormone deficiency associated with brain tumors. This case suggests that growth failure could be the only symptom in pediatric cases with brain tumors. Improved awareness regarding the association of growth failure with brain tumors is needed for earlier diagnosis and treatment. Furthermore, the growth curves should be carefully evaluated in regular health examinations at school.

摘要

在此,我们报告一例12岁女孩的病例,该女孩因身高明显低于中位数-5标准差以上而被转诊至我科。尽管她的生长发育迟缓在6岁时突然开始,但由于没有其他症状,她从未接受过检查。脑部MRI检查提示鞍上区有肿瘤,内分泌检查显示该肿瘤导致垂体激素联合缺乏。手术前,开始补充氢化可的松和左甲状腺素。手术切除肿瘤的病理诊断为黄色肉芽肿。她的生长曲线模式显示生长发育迟缓突然发作,这是垂体功能障碍继发身材矮小的典型模式,包括与脑肿瘤相关的生长激素缺乏。该病例表明,生长发育迟缓可能是小儿脑肿瘤病例的唯一症状。为了早期诊断和治疗,需要提高对生长发育迟缓与脑肿瘤关联的认识。此外,在学校定期健康检查中应仔细评估生长曲线。

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