2. Academic Validation
  3. Intrapericardial NUT Midline Carcinoma: Unusual Presentation of a Rare Tumor and Literature Review with Management Considerations

Intrapericardial NUT Midline Carcinoma: Unusual Presentation of a Rare Tumor and Literature Review with Management Considerations

  • Pediatr Cardiol. 2016 Jan;37(1):208-11. doi: 10.1007/s00246-015-1313-3.
Stefani Samples 1,  Katrina Gleditsch 2,  Anastasios Polimenakos 3
Affiliations

Affiliations

  • 1 Department of Pediatric Cardiology, Children's Hospital of Georgia, Medical College of Georgia, Georgia Regents University, Augusta, GA, USA. ssamples@gru.edu.
  • 2 Department of Pediatrics, Children's Hospital of Georgia, Medical College of Georgia, Georgia Regents University, Augusta, GA, USA.
  • 3 Department of Pediatric Cardiothoracic Surgery, Children's Hospital of Georgia, Medical College of Georgia, Georgia Regents University, Augusta, GA, USA.
PMID: 26667959 DOI: 10.1007/s00246-015-1313-3
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.

Keywords

Carcinoma; Cardiac tumor; Malignancy; Surgical resection.

Figures
Products