Orbital Sporadic Burkitt Lymphoma in an Adult Diabetic African American Female and a Review of Adult Orbital Cases

Overview

Journal Clin Ophthalmol

Publisher Dove Medical Press

Specialty Ophthalmology

Date 2011 May 17

PMID 21573040

Citations 3

Authors

John Carmody

Raghunath P Misra

Marlyn P Langford

William A Byrd

Lauren Ditta

Bryan Vekovius

Donald E Texada

Affiliations

Soon will be listed here.

Abstract

A case of sporadic Burkitt lymphoma (sBL) presenting with jaw and lid involvement in a diabetic adult African American female and a review of adult orbital Burkitt lymphoma cases are presented. Lid edema, visual loss, ophthalmoparesis, proptosis, and sinusitis progressed over 4 weeks despite antibiotic and steroid treatment. Upper lid biopsy histopathological evaluation and immunophenotyping revealed a homogenous mass of atypical CD10 and CD20-negative B-cells and tingible body macrophages yielding a "starry sky" appearance. Cytogenetic analysis detected a minor variant c-MYC translocation, but no Epstein-Barr virus RNA. Detection of multiple lesions prompted a diagnosis of stage IV disease that totally regressed following radiation and chemotherapy. Review results of the six adult orbital sBL cases support a poor prognosis and a heightened suspicion of variant CD10, CD20 and BCL6 positive sBL in adults presenting with jaw pain and rapidly progressive orbital symptoms, particularly in female, African American, and diabetic patients.

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References

Schubert S, Abdul-Khaliq H, Lehmkuhl H, Yegitbasi M, Reinke P, Kebelmann-Betzig C . Diagnosis and treatment of post-transplantation lymphoproliferative disorder in pediatric heart transplant patients. Pediatr Transplant. 2008; 13(1):54-62. DOI: 10.1111/j.1399-3046.2008.00969.x. View

Rosenzweig M, Rosenthal C, Torres V, Vaickus L . Development of a quantitative assay to measure EBV viral load in patients with autoimmune type 1 diabetes and healthy subjects. J Virol Methods. 2009; 164(1-2):111-5. DOI: 10.1016/j.jviromet.2009.11.018. View

Coupland S, Krause L, Delecluse H, Anagnostopoulos I, Foss H, Hummel M . Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases. Ophthalmology. 1998; 105(8):1430-41. DOI: 10.1016/S0161-6420(98)98024-1. View

Epstein M, ACHONG B, BARR Y . VIRUS PARTICLES IN CULTURED LYMPHOBLASTS FROM BURKITT'S LYMPHOMA. Lancet. 1964; 1(7335):702-3. DOI: 10.1016/s0140-6736(64)91524-7. View

Cader F, Kearns P, Young L, Murray P, Vockerodt M . The contribution of the Epstein-Barr virus to the pathogenesis of childhood lymphomas. Cancer Treat Rev. 2010; 36(4):348-53. DOI: 10.1016/j.ctrv.2010.02.011. View

BURKITT D, OCONOR G . Malignant lymphoma in African children. I. A clinical syndrome. Cancer. 1961; 14:258-69. DOI: 10.1002/1097-0142(196103/04)14:2<258::aid-cncr2820140206>3.0.co;2-7. View

Edelstein C, Shields J, Shields C, De Potter P, Eagle Jr R, Turtel L . Non-African Burkitt lymphoma presenting with oral thrush and an orbital mass in a child. Am J Ophthalmol. 1997; 124(6):859-61. DOI: 10.1016/s0002-9394(14)71714-3. View

Lee Harris N, Horning S . Burkitt's lymphoma--the message from microarrays. N Engl J Med. 2006; 354(23):2495-8. DOI: 10.1056/NEJMe068075. View

Neri A, Barriga F, Knowles D, Magrath I, Dalla-Favera R . Different regions of the immunoglobulin heavy-chain locus are involved in chromosomal translocations in distinct pathogenetic forms of Burkitt lymphoma. Proc Natl Acad Sci U S A. 1988; 85(8):2748-52. PMC: 280076. DOI: 10.1073/pnas.85.8.2748. View

10.

Brady G, MacArthur G, Farrell P . Epstein-Barr virus and Burkitt lymphoma. J Clin Pathol. 2007; 60(12):1397-402. PMC: 2095571. DOI: 10.1136/jcp.2007.047977. View

11.

Gutierrez M, Bhatia K, Barriga F, Diez B, Muriel F, de Andreas M . Molecular epidemiology of Burkitt's lymphoma from South America: differences in breakpoint location and Epstein-Barr virus association from tumors in other world regions. Blood. 1992; 79(12):3261-6. View

12.

Gariani K, Tran C, Philippe J . [Diabetes and cancer: an injurious association]. Rev Med Suisse. 2010; 6(252):1193-4, 1196-8. View

13.

Grote V, Becker S, Kaaks R . Diabetes mellitus type 2 - an independent risk factor for cancer?. Exp Clin Endocrinol Diabetes. 2010; 118(1):4-8. DOI: 10.1055/s-0029-1243193. View

14.

Magrath I . The pathogenesis of Burkitt's lymphoma. Adv Cancer Res. 1990; 55:133-270. DOI: 10.1016/s0065-230x(08)60470-4. View

15.

Wu D, Wood B, Dorer R, Fromm J . "Double-Hit" mature B-cell lymphomas show a common immunophenotype by flow cytometry that includes decreased CD20 expression. Am J Clin Pathol. 2010; 134(2):258-65. DOI: 10.1309/AJCP7YLDTJPLCE5F. View

16.

Coupland S, Hummel M, Stein H . Ocular adnexal lymphomas: five case presentations and a review of the literature. Surv Ophthalmol. 2002; 47(5):470-90. DOI: 10.1016/s0039-6257(02)00337-5. View

17.

Frank D, Cesarman E, Liu Y, Michler R, Knowles D . Posttransplantation lymphoproliferative disorders frequently contain type A and not type B Epstein-Barr virus. Blood. 1995; 85(5):1396-403. View

18.

Diehl S, Schmidlin H, Nagasawa M, van Haren S, Kwakkenbos M, Yasuda E . STAT3-mediated up-regulation of BLIMP1 Is coordinated with BCL6 down-regulation to control human plasma cell differentiation. J Immunol. 2008; 180(7):4805-15. PMC: 2396731. DOI: 10.4049/jimmunol.180.7.4805. View

19.

Ziegler J . Burkitt's lymphoma. N Engl J Med. 1981; 305(13):735-45. DOI: 10.1056/NEJM198109243051305. View

20.

Shields J, Shields C, Scartozzi R . Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology. 2004; 111(5):997-1008. DOI: 10.1016/j.ophtha.2003.01.002. View