Life-threatening paraneoplastic syndrome in a child with sarcoma of the liver cured by emergency resection

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

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)153-155
Number of pages3
JournalJournal of pediatric hematology/oncology
Volume35
Issue number2
DOIs
StatePublished - Mar 1 2013

Keywords

  • Torsades de pointes
  • embryonal/germ cell neoplasm
  • liver neoplasm
  • long QT syndrome
  • paraneoplastic syndrome
  • sarcoma

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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