JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, vol.35, no.2, pp.153-155, 2013 (SCI-Expanded)
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.