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


Fricchione M. J. , Glenn N., Follmer R., Kent P. M.

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, vol.35, no.2, pp.153-155, 2013 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 35 Issue: 2
  • Publication Date: 2013
  • Doi Number: 10.1097/mph.0b013e318266bf55
  • Title of Journal : JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
  • Page Numbers: pp.153-155

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.