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Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e318266bf55
Clinical and Laboratory Observations

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*

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Abstract

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.

© 2013 Lippincott Williams & Wilkins, Inc.

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