Non-Hodgkin B-cell Lymphoma of the Ovary in a Child with Ataxia-Telangiectasia

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• 作者：C.S. Danby ; MD¹ ; L. Allen ; MD ; FRCSC² ; M.D. Moharir ; MD ; MSc ; FRACP³ ; S. Weitzman ; MB ; BCh ; FCP(SA) ; FRCP(C)⁴ ; T. Dumont ; MD ; FRCSC⁵ ; ^{tdumont@cheo.on.ca}
• 关键词：Ataxia Telangiectasia ; Ovarian Lymphoma ; Alpha-fetoprotein ; AFP
• 刊名：Journal of Pediatric and Adolescent Gynecology
• 出版年：2013
• 出版时间：April, 2013
• 年：2013
• 卷：26
• 期：2
• 页码：e43-e45
• 全文大小：67 K

文摘

Background

Ataxia-telangiectasia is a multisystem, life-limiting, recessively inherited genetic disorder caused by mutations in the Ataxia-telangiectasia mutated gene. It is characterized by the onset of changes in neurological and immunological development, organ maturation in childhood, as well as a high incidence of malignancies.

Case

We describe a case of an 11-year-old girl with a history of progressive ataxia and new finding of bilateral pelvic masses. Given an elevated alpha-fetoprotein, the pre-operative working diagnosis was a malignant germ cell tumor. Final ovarian pathology revealed a non-Hodgkin B-cell lymphoma with Burkitt-like morphology.

Summary

We present the first case of a primary ovarian non-Hodgkin B-cell lymphoma in a child with Ataxia-telangiectasia.