title: Intrapericardial NUT Midline Carcinoma: Unusual Presentation of a Rare Tumor and Literature Review with Management Considerations.
abstract: Rearrangements in the nuclear protein in testis (NUT) gene cause carcinomas that represent a rare but aggressive tumor type that often present at advanced stages in midline structures. Survival rarely exceeds 12 months from the time of diagnosis. There have been no reports of a primary cardiac presentation, and few studies have reported on the numerous treatment strategies. Given their aggressive and invasive nature, NUT midline carcinomas present a therapeutic dilemma. Treatment may include surgical resection, chemotherapy, or radiotherapy, but no consistently successful treatment has been established. Surgical resection is indicated to reduce symptomatic mass effect whenever present. Novel therapies with bromodomain extra-terminal inhibitors may be associated with potential survival benefit. Here, we describe an unusual presentation of this tumor. Literature review with management considerations is underlying.
authors.affiliation: Department of Pediatric Cardiology, Children's Hospital of Georgia, Medical College of Georgia, Georgia Regents University, Augusta, GA, USA. ssamples@gru.edu.
authors.forename: Stefani
authors.initials: S
authors.lastname: Samples
authors.affiliation: Department of Pediatrics, Children's Hospital of Georgia, Medical College of Georgia, Georgia Regents University, Augusta, GA, USA.
authors.forename: Katrina
authors.initials: K
authors.lastname: Gleditsch
authors.affiliation: Department of Pediatric Cardiothoracic Surgery, Children's Hospital of Georgia, Medical College of Georgia, Georgia Regents University, Augusta, GA, USA.
authors.forename: Anastasios
authors.initials: A
authors.lastname: Polimenakos
mesh_terms: D002277:Carcinoma / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D002675:Child, Preschool; D005260:Female; D006338:Heart Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D006801:Humans; D009363:Neoplasm Proteins; D009687:Nuclear Proteins / Q000235:genetics*; D015513:Oncogene Proteins / Q000235:genetics*; D014057:Tomography, X-Ray Computed
chemical_list: C000630260:NUTM1 protein, human; D009363:Neoplasm Proteins; D009687:Nuclear Proteins; D015513:Oncogene Proteins
keywords: Carcinoma; Cardiac tumor; Malignancy; Surgical resection