Metachronous T‐Lymphoblastic Lymphoma and Burkitt Lymphoma in a Child With Constitutional Mismatch Repair Deficiency Syndrome. Issue 8 (1st April 2016)

• Record Type: Journal Article
• Authors: Alexander, Thomas B.; McGee, Rose B.; Kaye, Erica C.; McCarville, Mary Beth; Choi, John K.; Cavender, Cary P.; Nichols, Kim E.; Sandlund, John T.
• Is Part Of: Pediatric blood & cancer. Volume 63:Issue 8(2016)
• Page Start: 1454
• Page End: 1456
• Publication Date: 2016-04-01
• Subjects: hereditary -- lymphoma -- oncology -- predisposition; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.25989
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract : Constitutional mismatch repair deficiency (CMMRD) is a cancer predisposition syndrome associated with a high risk of developing early‐onset malignancies of the blood, brain, and intestinal tract. We present the case of a patient with T‐lymphoblastic lymphoma at the age of 3 years, followed by Burkitt lymphoma 10 years later. This patient also exhibited numerous nonmalignant findings including café au lait spots, lipomas, bilateral renal nodules, a nonossifying fibroma, multiple colonic adenomas, and a rapidly enlarging pilomatrixoma. The spectrum of malignant and nonmalignant neoplasms in this patient highlights the remarkable diversity, and early onset, of lesions seen in children with CMMRD.