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

doi:10.1097/MPH.0b013e318266bf55

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

Pediatrics

Research output: Contribution to journal › Article › peer-review

4 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 language	English (US)
Pages (from-to)	153-155
Number of pages	3
Journal	Journal of pediatric hematology/oncology
Volume	35
Issue number	2
DOIs	https://doi.org/10.1097/MPH.0b013e318266bf55
State	Published - Mar 2013

Keywords

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

ASJC Scopus subject areas

Hematology
Oncology
Pediatrics, Perinatology, and Child Health

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1097/MPH.0b013e318266bf55

Cite this

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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.",

keywords = "Torsades de pointes, embryonal/germ cell neoplasm, liver neoplasm, long QT syndrome, paraneoplastic syndrome, sarcoma",

author = "Fricchione, {Marielle J.} and Nicole Glenn and Rachel Follmer and Kent, {Paul M.}",

year = "2013",

month = mar,

doi = "10.1097/MPH.0b013e318266bf55",

language = "English (US)",

volume = "35",

pages = "153--155",

journal = "Journal of pediatric hematology/oncology",

issn = "1077-4114",

publisher = "Lippincott Williams and Wilkins",

number = "2",

}

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T1 - Life-threatening paraneoplastic syndrome in a child with sarcoma of the liver cured by emergency resection

AU - Fricchione, Marielle J.

AU - Glenn, Nicole

AU - Follmer, Rachel

AU - Kent, Paul M.

PY - 2013/3

Y1 - 2013/3

N2 - 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.

AB - 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.

KW - Torsades de pointes

KW - embryonal/germ cell neoplasm

KW - liver neoplasm

KW - long QT syndrome

KW - paraneoplastic syndrome

KW - sarcoma

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AN - SCOPUS:84874484211

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JF - Journal of pediatric hematology/oncology

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ER -

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

Abstract

Keywords

ASJC Scopus subject areas

UN SDGs

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