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DiGeorge syndrome who developed lymphoproliferative mediastinal mass

 Kyu Yeun Kim  ;  Ji Ae Hur  ;  Ki Hwan Kim  ;  Yoon Jin Cha  ;  Mi Jung Lee  ;  Dong Soo Kim 
 Korean Journal of Pediatrics, Vol.58(3) : 108-111, 2015 
Journal Title
Korean Journal of Pediatrics
Issue Date
DiGeorge syndrome ; Lymphoproliferative disorders ; Mediastinal neoplasms
DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing thymic shadow on their plain chest x-ray. Immunodeficient patients are traditionally known to be at an increased risk for malignancy, especially lymphoma. We experienced a 7-year-old DiGeorge syndrome patient with mediastinal mass shadow on her plain chest x-ray. She visited Severance Children's Hospital hospital with recurrent pneumonia, and throughout her repeated chest x-ray, there was a mass like shadow on anterior mediastinal area. We did full evaluation including chest computed tomography, chest ultrasonography, and chest magnetic resonance imaging. To rule out malignancy, video assisted thoracoscopic surgery was done. Final diagnosis of the mass which was thought to be malignancy, was lymphoproliferative lesion.
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1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Radiology (영상의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Ki Hwan(김기환)
Kim, Dong Soo(김동수)
Lee, Mi-Jung(이미정) ORCID logo https://orcid.org/0000-0003-3244-9171
Cha, Yoon Jin(차윤진) ORCID logo https://orcid.org/0000-0002-5967-4064
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