title: Rare Presentations of Epstein-Barr Virus--Associated Smooth Muscle Tumor in Children.
abstract: Epstein-Barr virus (EBV) has oncogenic potential and has been implicated in the etiology of a wide range of malignancies. Certain EBV-driven neoplasms, such as smooth muscle tumors (SMTs), manifest typically in immunocompromised patients. In children, these neoplasms have been encountered in the setting of primary immune disorders, specifically severe combined and common variable immunodeficiency syndromes. Human immunodeficiency virus infection and posttransplant immunosuppression, in particular liver and kidney transplantation, likewise increase the risk in the pediatric population. The location of these neoplasms appears related to the type of immunodeficiency: in acquired immunodeficiency syndrome they are frequently located intracranially or intraspinally, whereas after transplant they usually involve the liver or lung. We report 2 distinct cases of EBV-related SMT, unique through their coassociated immunosuppressive state or location: the 1st occurred in a patient with immunodeficiency secondary to NEMO gene mutation following hematopoietic stem cell transplantation; the 2nd developed in the orbit after heart transplant.
authors.affiliation: Department of Pathology and Laboratory Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL 60611, USA.
authors.forename: Nicoleta C
authors.initials: NC
authors.lastname: Arva
authors.affiliation: Department of Pathology and Laboratory Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL 60611, USA.
authors.forename: Kristian T
authors.initials: KT
authors.lastname: Schafernak
mesh_terms: D000199:Actins / Q000032:analysis; D000328:Adult; D001344:Autopsy; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D004476:Ectodermal Dysplasia / Q000235:genetics / Q000276:immunology / Q000601:surgery; D005134:Eye Neoplasms / Q000276:immunology / Q000473:pathology / Q000628:therapy / Q000821:virology*; D017809:Fatal Outcome; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000235:genetics / Q000276:immunology / Q000601:surgery; D016027:Heart Transplantation / Q000009:adverse effects; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000276:immunology / Q000302:isolation & purification*; D006801:Humans; D016867:Immunocompromised Host; D007150:Immunohistochemistry; D007153:Immunologic Deficiency Syndromes / Q000235:genetics / Q000276:immunology / Q000601:surgery; D007166:Immunosuppressive Agents / Q000009:adverse effects; D017403:In Situ Hybridization; D007223:Infant; D008297:Male; D000081207:Primary Immunodeficiency Diseases; D012367:RNA, Viral / Q000235:genetics; D012307:Risk Factors; D018235:Smooth Muscle Tumor / Q000276:immunology / Q000473:pathology / Q000628:therapy / Q000821:virology*; D013160:Splenic Neoplasms / Q000276:immunology / Q000473:pathology / Q000628:therapy / Q000821:virology*
chemical_list: C541116:ACTA2 protein, human; D000199:Actins; D014408:Biomarkers, Tumor; D007166:Immunosuppressive Agents; D012367:RNA, Viral
keywords: Epstein-Barr virus–associated smooth muscle tumor; immunosuppression; transplant