title: Life-threatening paraneoplastic syndrome in a child with sarcoma of the liver cured by emergency resection.
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.
authors.affiliation: Chicago Medical School, Chicago, IL, USA. marielle_fricchione@rush.edu
authors.forename: Marielle J
authors.initials: MJ
authors.lastname: Fricchione
authors.forename: Nicole
authors.initials: N
authors.lastname: Glenn
authors.forename: Rachel
authors.initials: R
authors.lastname: Follmer
authors.forename: Paul M
authors.initials: PM
authors.lastname: Kent
mesh_terms: D002648:Child; D004630:Emergencies; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications* / Q000601:surgery; D008133:Long QT Syndrome / Q000209:etiology*; D010257:Paraneoplastic Syndromes / Q000209:etiology*; D012509:Sarcoma / Q000150:complications* / Q000601:surgery; D016171:Torsades de Pointes / Q000209:etiology*