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儿童期原发性中枢神经系统淋巴瘤表现为进行性全垂体功能减退。

Primary central nervous system lymphoma in childhood presenting as progressive panhypopituitarism.

作者信息

Silfen M E, Garvin J H, Hays A P, Starkman H S, Aranoff G S, Levine L S, Feldstein N A, Wong B, Oberfield S E

机构信息

Division of Pediatric Endocrinology, Columbia-Presbyterian Medical Center, New York, New York 10032, USA.

出版信息

J Pediatr Hematol Oncol. 2001 Feb;23(2):130-3. doi: 10.1097/00043426-200102000-00013.

DOI:10.1097/00043426-200102000-00013
PMID:11216706
Abstract

We report a 15-year-old boy who had isolated central diabetes insipidus initially diagnosed at age 11 years. A brain magnetic resonance imaging (MRI) was normal at the time. At age 12 years, growth hormone (GH) testing was performed because of a decline in linear growth rate and demonstrated GH deficiency. After a repeat normal brain MRI, GH therapy was begun. Three years later, hormonal testing revealed prepubertal gonadotropins and low testosterone levels, free thyroxine index, and morning cortisol levels. Repeat brain MRI demonstrated a 9-mm enhancing lesion in the region of the pituitary stalk. The pathologic diagnosis was that of a high-grade malignant B-cell lymphoma, suggestive of Burkitt Lymphoma. Growth hormone therapy has not been associated with an increased incidence of lymphoma. This report underscores the need for vigilance in follow-up brain imaging and hormonal evaluation in children with diabetes insipidus, especially those with evolving anterior hormone deficiencies.

摘要

我们报告一名15岁男孩,他最初在11岁时被诊断为孤立性中枢性尿崩症。当时脑部磁共振成像(MRI)正常。12岁时,由于线性生长速率下降进行了生长激素(GH)检测,结果显示生长激素缺乏。在重复脑部MRI检查正常后,开始进行生长激素治疗。三年后,激素检测显示青春期前促性腺激素、低睾酮水平、游离甲状腺素指数和早晨皮质醇水平异常。重复脑部MRI显示垂体柄区域有一个9毫米的强化病变。病理诊断为高级别恶性B细胞淋巴瘤,提示伯基特淋巴瘤。生长激素治疗与淋巴瘤发病率增加无关。本报告强调了对尿崩症患儿,尤其是那些出现垂体前叶激素缺乏进展的患儿进行脑部影像学随访和激素评估时保持警惕的必要性。

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