Hindawi Publishing Corporation Case Reports in Medicine Volume 2011, Article ID 327263, 4 pages doi:10.1155/2011/327263 Case Report Bilateral Burkitt Lymphoma of the Ovaries: A Report of a Case in a Child with Williams Syndrome Grace Ifeyinwa Onimoe,1, 2 Samir Kahwash,1, 3 Amanda Termuhlen,1, 2 Thomas G. Gross,1, 2 Elizabeth Varga,1, 2 and Melissa J. Rose1, 2 1 Department of Pediatrics, College of Medicine, The Ohio State University, Columbus, OH 43210, USA 2 Department of Pediatric Hematology-Oncology, Nationwide Children’ Hospital, Columbus, OH 43205, USA 3 Department of Pathology, College of Medicine, The Ohio State University, Columbus, OH 43210, USA Correspondence should be addressed to Melissa J. Rose, melissa.rose@nationwidechildrens.org Received 31 January 2011; Revised 29 March 2011; Accepted 4 April 2011 Academic Editor: Cherie H. Dunphy Copyright © 2011 Grace Ifeyinwa Onimoe et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. A 10-year-old female with Williams Syndrome (WS) presented with a two-month history of fatigue, weight loss, and bilateral ovarian masses. Histologic, immunophenotypic, and cytogenetic studies conﬁrmed the diagnosis of Burkitt lymphoma (BL). While there is no established association between the two disorders, this is the third case in the literature of Burkitt lymphoma in a patient with Williams Syndrome. 1. Introduction Although relatively rare in adults, Burkitt lymphoma consti- tutes more than 40% of pediatric non-Hodgkin lymphomas (NHL). The World Health Organization (WHO) classiﬁca- tion (2008) recognizes three clinical subtypes: endemic, spo- radic, and immunodeﬁciency associated. In the Western Hemisphere, most cases present in the gastrointestinal tract. Cases involving the jaw, kidneys, breasts, and other sites are common, including bilateral involvement [1]. Williams Syndrome (also known as Williams-Beuren Syndrome) is a rare genetic disorder with an incidence of one per 7,500–20,000 births; most cases occurring sporadically. Clinical characteristics of Williams Syndrome are growth and mental retardation with a friendly, outgoing personality, dysmorphic facial features, hypercalcemia in early infancy, and congenital cardiovascular malformations, in particular supravalvular aortic stenosis [2]. Williams Syndrome is the consequence of an interstitial microdeletion at 7q11.23, which includes the elastin gene producing hemizygosity at the elastin gene locus. We describe the third case of Burkitt lymphoma in a child with Williams Syndrome [3, 4]. 2. Case Presentation A 10-year-old female diagnosed with Williams Syndrome at age 9 years, conﬁrmed by ﬂuorescent in situ hybridization (FISH), presented with a two-month history of fatigue, de- creased activity, weight loss, regression in bladder control, and a recently palpated pelvic mass. Her medical history was also signiﬁcant for congenital hypothyroidism and aortic valvular stenosis. Physical examination showed WS facial dysmorphia, murmur consistent with aortic valvular stenosis, mild hyper- tension, and a 2.5 cm by 2.5 cm protruding mass just me- dial to the left anterior superior iliac spine, without deﬁned edges. Abdominal CT study revealed four discrete masses: two large masses within the pelvis causing bilateral hydronephro- sis, a third involving the hilum of the left kidney interfering with arterial and venous blood ﬂow of the kidney, and a fourth involving the head of the pancreas. Metastasis to the left femoral diaphyses and proximal left humerus was also noted. Laboratory evaluation demonstrated a progressively elevated LDH, normal uric acid, BUN, and creatinine. Sero- logical workup for Epstein Barr Virus and cytomegalovirus