TY - JOUR
T1 - Intrapericardial NUT Midline Carcinoma
T2 - Unusual Presentation of a Rare Tumor and Literature Review with Management Considerations
AU - Samples, Stefani
AU - Gleditsch, Katrina
AU - Polimenakos, Anastasios
PY - 2016/1/1
Y1 - 2016/1/1
N2 - 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.
AB - 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.
KW - Carcinoma
KW - Cardiac tumor
KW - Malignancy
KW - Surgical resection
UR - http://www.scopus.com/inward/record.url?scp=84957438200&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84957438200&partnerID=8YFLogxK
U2 - 10.1007/s00246-015-1313-3
DO - 10.1007/s00246-015-1313-3
M3 - Article
C2 - 26667959
AN - SCOPUS:84957438200
SN - 0172-0643
VL - 37
SP - 208
EP - 211
JO - Pediatric Cardiology
JF - Pediatric Cardiology
IS - 1
ER -