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, cilt.35, ss.153-155, 2013 (SCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 35 Konu: 2
  • Basım Tarihi: 2013
  • Doi Numarası: 10.1097/mph.0b013e318266bf55
  • Dergi Adı: JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
  • Sayfa Sayıları: ss.153-155

Özet

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.