| Literature DB >> 26667959 |
Stefani Samples1, Katrina Gleditsch2, Anastasios Polimenakos3.
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.Entities:
Keywords: Carcinoma; Cardiac tumor; Malignancy; Surgical resection
Mesh:
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Year: 2015 PMID: 26667959 DOI: 10.1007/s00246-015-1313-3
Source DB: PubMed Journal: Pediatr Cardiol ISSN: 0172-0643 Impact factor: 1.655