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Life-threatening Paraneoplastic Syndrome in a Child With Sarcoma of the Liver Cured by Emergency Resection

Fricchione, Marielle J. MD; Glenn, Nicole MD; Follmer, Rachel MD; Kent, Paul M. MD

Journal of Pediatric Hematology/Oncology: March 2013 - Volume 35 - Issue 2 - p 153–155
doi: 10.1097/MPH.0b013e318266bf55
Clinical and Laboratory Observations

We believe this to be the first case of life-threatening paraneoplastic syndrome (PNS) in a child with undifferentiated embryonal sarcoma of the liver (UESL). We report a case of a 9-year-old child with UESL believed to be unresectable at presentation, who experienced life-threatening, refractory long QTc syndrome, Torsades de pointes, fevers of unknown origin, and secretory diarrhea necessitating emergency surgery. After failure of intravenous immunoglobulin and high-dose steroids to control PNS and despite pancytopenia from chemotherapy, very aggressive surgical resection was life saving and led to rapid resolution of PNS.

*Rush University Medical Center

Chicago Medical School, Chicago, IL

The authors declare no conflict of interest.

Reprints: Marielle J. Fricchione, MD, 1653 W. Congress Parkway, Suite 710, Chicago, IL 60612 (e-mail:

Received September 14, 2011

Accepted June 26, 2012

Copyright © 2013 Wolters Kluwer Health, Inc. All rights reserved.