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Mucosal-associated Lymphoid Tissue (MALT) Lymphoma in Association With Pediatric Primary Sjogren Syndrome

2 Cases and Review

Tesher, Melissa S. MD, MS*; Esteban, Ysabella MD*; Henderson, Tara O. MD*; Villanueva, Gabriela MD*; Onel, Karen B. MD

Journal of Pediatric Hematology/Oncology: October 26, 2018 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPH.0000000000001321
Clinical and Laboratory Observations: PDF Only

Mucosal-associated lymphoid tissue (MALT) lymphoma is rare in the pediatric population, but primary Sjogren syndrome is a well-established risk factor for this malignancy. This report describes 2 cases of MALT lymphoma in children with Sjogren syndrome. A 15-year-old girl developed MALT lymphoma of the parotid gland as the presenting symptom of Sjogren syndrome. In the second case, a 15-year-old boy with known Sjogren syndrome presenting mainly with arthritis was diagnosed with MALT lymphoma, also of the parotid gland. With early diagnosis and treatment, outcomes in pediatric MALT lymphoma are generally encouraging. Pediatric oncology specialists should be aware of the association of MALT lymphoma with Sjogren syndrome and have a high index of suspicion for this malignant complication.

*University of Chicago Medical Center, Chicago, IL

Hospital for Special Surgery, Weill Cornell Medicine, New York, NY

The authors declare no conflict of interest.

Reprints: Melissa S. Tesher, MD, MS, University of Chicago Medical Center, 5841 South Maryland Avenue, MC 5044, Chicago, IL 60637 (e-mail:

Received February 6, 2018

Accepted August 22, 2018

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