PMID- 22033856
OWN - NLM
STAT- MEDLINE
DCOM- 20120619
LR  - 20211203
IS  - 1432-1998 (Electronic)
IS  - 0301-0449 (Linking)
VI  - 42
IP  - 2
DP  - 2012 Feb
TI  - NUT midline carcinoma: an imaging case series and review of literature.
PG  - 205-10
LID - 10.1007/s00247-011-2272-3 [doi]
AB  - BACKGROUND: NUT midline carcinoma is a rare and aggressive tumor that has 
      primarily been reported in children and young adults. The tumor is characterized 
      by a rearrangement on the nuclear protein in testis (NUT) gene, located on 
      chromosome 15q14, resulting in the BRD4-NUT fusion oncogene. This carcinoma most 
      commonly presents in the midline and displays an invariably lethal clinical 
      course. OBJECTIVE: To highlight the imaging features of NUT midline carcinoma. 
      MATERIALS AND METHODS: IRB approval was obtained for chart review. We 
      retrospectively reviewed the chart and imaging studies of three children. All 
      three cases were diagnosed by karyotyping and confirmed by fluorescence in situ 
      hybridization (FISH). Cross-sectional imaging including CT, MRI and, in one 
      patient, PET was available for review. RESULTS: Two out of three children 
      presented with midline and multifocal disease. The third case had a medial left 
      thigh mass and no metastatic disease at initial presentation. The common imaging 
      features include heterogeneous low density on CT and T1 hypointensity and 
      low-level T2 hyperintensity on MRI with heterogeneous enhancement. All cases were 
      confirmed pathologically and by molecular studies. CONCLUSION: NUT midline 
      carcinoma usually presents in the midline, either in the head, neck or chest. We 
      present three cases with the bulk of the tumor below the diaphragm, which is seen 
      in the minority of patients with NUT midline carcinoma, according to the 
      available literature. Metastatic disease is common at initial presentation and 
      can be quite extensive. The most striking feature of this disease is its 
      aggressive nature with exponential interval growth of tumor.
FAU - Polsani, Aruna
AU  - Polsani A
AD  - Department of Radiology & Imaging Sciences, Children's Healthcare of Atlanta, 
      Emory University, 1405 Clifton Road NE, Atlanta, GA 30322, USA. 
      aruna_polsani@yahoo.com
FAU - Braithwaite, Kiery A
AU  - Braithwaite KA
FAU - Alazraki, Adina L
AU  - Alazraki AL
FAU - Abramowsky, Carlos
AU  - Abramowsky C
FAU - Shehata, Bahig M
AU  - Shehata BM
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Review
DEP - 20111028
PL  - Germany
TA  - Pediatr Radiol
JT  - Pediatric radiology
JID - 0365332
RN  - 0 (Contrast Media)
RN  - 0 (NUTM1 protein, human)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (Nuclear Proteins)
RN  - 0 (Oncogene Proteins)
SB  - IM
MH  - Adolescent
MH  - Carcinoma/*diagnosis
MH  - Contrast Media
MH  - Diagnosis, Differential
MH  - Fatal Outcome
MH  - Female
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Infant
MH  - Infant, Newborn
MH  - Karyotype
MH  - Liver Neoplasms/*diagnosis
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Neoplasm Proteins
MH  - Nuclear Proteins
MH  - Oncogene Proteins
MH  - Orbital Neoplasms/*diagnosis
MH  - Positron-Emission Tomography
MH  - Retrospective Studies
MH  - *Thigh
MH  - Tomography, X-Ray Computed
EDAT- 2011/10/29 06:00
MHDA- 2012/06/20 06:00
CRDT- 2011/10/29 06:00
PHST- 2011/06/23 00:00 [received]
PHST- 2011/09/12 00:00 [accepted]
PHST- 2011/09/02 00:00 [revised]
PHST- 2011/10/29 06:00 [entrez]
PHST- 2011/10/29 06:00 [pubmed]
PHST- 2012/06/20 06:00 [medline]
AID - 10.1007/s00247-011-2272-3 [doi]
PST - ppublish
SO  - Pediatr Radiol. 2012 Feb;42(2):205-10. doi: 10.1007/s00247-011-2272-3. Epub 2011 
      Oct 28.