2013 Apr;26(2):e43-5. doi: 10.1016/j.jpag.2012.09.003. Epub 2013 Jan 9.

Author information

1
Department of Obstetrics and Gynecology, Maine Medical Center, Portland, ME, USA.

Abstract

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.

PMID:
 
23312583
 
DOI:
 
10.1016/j.jpag.2012.09.003
[Indexed for MEDLINE]