PMID- 32153680
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200928
IS  - 1878-5409 (Electronic)
IS  - 1878-5409 (Linking)
VI  - 21
IP  - 3
DP  - 2020 Mar
TI  - Intimal sarcoma of the left atrium presenting with transient ischaemic attack - A 
      case report and review of the literature.
PG  - 89-92
LID - 10.1016/j.jccase.2019.10.006 [doi]
AB  - A 31-year-old female with a history of polycystic ovary syndrome and two recent 
      miscarriages presented with symptoms of a transient ischemic attack. 
      Echocardiography to assess for possible embolic source identified a 4.8 cm left 
      atrial mass, presumed to be an atrial myxoma. At surgery the tumor was suspicious 
      of malignancy. Histopathology revealed a heterogeneous hyper- and hypo-cellular 
      spindle cell tumor showing mild atypia. Atrial myxoma markers were negative. The 
      Ki67 proliferation factor was 30% and fluorescence in situ hybridization (FISH) 
      analysis showed MDM2 amplification. Expert review confirmed the morphological, 
      immunohistochemical, and FISH features to be of a cardiac intimal sarcoma. Recent 
      improvements in imaging, surgery, and molecular testing have increased diagnoses 
      of primary cardiac intimal sarcomas. Here we discuss the pathological and 
      clinical implications of these rare atrial myxoma mimics. <Learning objective: 
      Intimal sarcomas are rare, aggressive tumors typically associated with great 
      vessels, however, molecular diagnoses of cardiac intimal sarcomas are increasing. 
      Presentation is varied. Cardiac symptoms and emboli in young patients should 
      prompt investigation with high levels of suspicion. Intimal sarcomas show MDM2 
      amplification with genetic aberration. Careful pre-operative planning to achieve 
      clear surgical margins almost doubles life expectancy. Chemoradiotherapy may be 
      beneficial. MDM2 and PDGFRa inhibitors are in development.>.
CI  - Crown Copyright (c) 2019 Published by Elsevier Ltd on behalf of Japanese College of 
      Cardiology. All rights reserved.
FAU - Grant, Lucy
AU  - Grant L
AD  - Black Country Pathology Services, New Cross Hospital, Wolverhampton, UK.
FAU - Morgan, Ian
AU  - Morgan I
AD  - New Cross Hospital, Wolverhampton, UK.
FAU - Sumathi, VaiyaPuri
AU  - Sumathi V
AD  - Royal Orthopaedic Hospital, Birmingham, UK.
FAU - Salmons, Nabeel
AU  - Salmons N
AD  - Black Country Pathology Services, New Cross Hospital, Wolverhampton, UK.
LA  - eng
PT  - Case Reports
DEP - 20191116
PL  - Japan
TA  - J Cardiol Cases
JT  - Journal of cardiology cases
JID - 101549579
PMC - PMC7054658
OTO - NOTNLM
OT  - Atrial
OT  - Cardiac
OT  - Intimal sarcoma
OT  - MDM2
OT  - Mesenchymal
EDAT- 2020/03/11 06:00
MHDA- 2020/03/11 06:01
PMCR- 2019/11/16
CRDT- 2020/03/11 06:00
PHST- 2019/05/14 00:00 [received]
PHST- 2019/09/07 00:00 [revised]
PHST- 2019/10/07 00:00 [accepted]
PHST- 2020/03/11 06:00 [entrez]
PHST- 2020/03/11 06:00 [pubmed]
PHST- 2020/03/11 06:01 [medline]
PHST- 2019/11/16 00:00 [pmc-release]
AID - S1878-5409(19)30106-9 [pii]
AID - 10.1016/j.jccase.2019.10.006 [doi]
PST - epublish
SO  - J Cardiol Cases. 2019 Nov 16;21(3):89-92. doi: 10.1016/j.jccase.2019.10.006. 
      eCollection 2020 Mar.