Childhood cancer predisposition syndromes-A concise review and recommendations by the Cancer Predisposition Working Group of the Society for Pediatric Oncology and Hematology.
Heritable predisposition is an important cause of cancer in children and adolescents. Although a large number of cancer predisposition genes and their associated syndromes and malignancies have already been described, it appears likely that there are more pediatric cancer patients in whom heritable cancer predisposition syndromes have yet to be recognized. In a consensus meeting in the beginning of 2016, we convened experts in Human Genetics and Pediatric Hematology/Oncology to review the available data, to categorize the large amount of information, and to develop recommendations regarding when a cancer predisposition syndrome should be suspected in a young oncology patient. This review summarizes the current knowledge of cancer predisposition syndromes in pediatric oncology and provides essential information on clinical situations in which a childhood cancer predisposition syndrome should be suspected.
Department of Human Genetics, Hannover Medical School, Hannover, Germany.
Tim
T
Ripperger
Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart-Olgahospital, Stuttgart, Germany.
Stefan S
SS
Bielack
Medical Faculty, Department of Pediatric Oncology, Hematology and Clinical Immunology, University Children's Hospital, Heinrich Heine University, Düsseldorf, Germany.
Arndt
A
Borkhardt
General Pediatrics, Hematology/Oncology, University Children's Hospital Tuebingen, Tuebingen, Germany.|Department of Pediatrics and Adolescent Medicine, University of Erlangen-Nuremberg, Erlangen, Germany.
Ines B
IB
Brecht
Pediatric Hematology and Oncology, University Children's Hospital Muenster, Muenster, Germany.
Birgit
B
Burkhardt
Pediatric Hematology and Oncology, University Children's Hospital Muenster, Muenster, Germany.
Gabriele
G
Calaminus
Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany.
Klaus-Michael
KM
Debatin
Department of Pediatric Oncology and Hematology, Charité University Medicine, Berlin, Germany.
Hedwig
H
Deubzer
Pediatric Hematology and Oncology, University Children's Hospital Muenster, Muenster, Germany.
Uta
U
Dirksen
Department of Pediatric Oncology and Hematology, Charité University Medicine, Berlin, Germany.
Cornelia
C
Eckert
Department of Pediatric Oncology and Hematology, Charité University Medicine, Berlin, Germany.
Angelika
A
Eggert
Faculty of Medicine, Division of Pediatric Hematology and Oncology Medical Center, Department of Pediatrics and Adolescent Medicine, University of Freiburg, Freiburg, Germany.
Miriam
M
Erlacher
Pediatric Oncology and Hematology, Pediatrics III, University Hospital of Essen, Essen, Germany.
Gudrun
G
Fleischhack
Children's Hospital Augsburg, Swabian Children's Cancer Center, Augsburg, Germany.
Michael C
MC
Frühwald
Children's Hospital Augsburg, Swabian Children's Cancer Center, Augsburg, Germany.
Astrid
A
Gnekow
Department of Human Genetics, Hannover Medical School, Hannover, Germany.
Gudrun
G
Goehring
Department of Pediatric Hematology and Oncology, University of Saarland, Homburg, Germany.
Norbert
N
Graf
Medical Faculty, Department of Pediatric Oncology, Hematology and Clinical Immunology, University Children's Hospital, Heinrich Heine University, Düsseldorf, Germany.|Department of Otorhinolaryngology and Head/Neck Surgery, Heinrich Heine University, Düsseldorf, Germany.
Helmut
H
Hanenberg
Medical Faculty, Department of Pediatric Oncology, Hematology and Clinical Immunology, University Children's Hospital, Heinrich Heine University, Düsseldorf, Germany.
Julia
J
Hauer
Department of Pediatric Hematology and Oncology, University of Cologne, Cologne, Germany.
Barbara
B
Hero
Faculty of Medicine, Division of Pediatric Hematology and Oncology Medical Center, Department of Pediatrics and Adolescent Medicine, University of Freiburg, Freiburg, Germany.
Simone
S
Hettmer
Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Katja
K
von Hoff
Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Martin
M
Horstmann
Institute of Human Genetics, University of Erlangen-Nuremberg, Erlangen, Germany.
Juliane
J
Hoyer
Department of Human Genetics, Hannover Medical School, Hannover, Germany.|Hannover Unified Biobank, Hannover Medical School, Hannover, Germany.
Thomas
T
Illig
German Childhood Cancer Registry (GCCR), Institute for Medical Biostatistics, Epidemiology and Informatics (IMBEI), University Medical Center of the Johannes Gutenberg-University Mainz, Mainz, Germany.
Peter
P
Kaatsch
Department of Pediatric Surgery, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University, Munich, Germany.
Roland
R
Kappler
Pediatric Hematology and Oncology, University Children's Hospital Muenster, Muenster, Germany.
Kornelius
K
Kerl
Hospital for Children and Adolescents, Goethe-University Frankfurt, Frankfurt am Main, Germany.
Thomas
T
Klingebiel
Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University Medical Center, Aachen, Germany.
Udo
U
Kontny
Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Uwe
U
Kordes
Department of Pediatric Hematology and Oncology, Justus Liebig University, Giessen, Germany.
Dieter
D
Körholz
Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart-Olgahospital, Stuttgart, Germany.
Ewa
E
Koscielniak
Division of Pediatric Hematology and Oncology, University Medical Center Goettingen, Goettingen, Germany.
Christof M
CM
Kramm
Medical Faculty, Department of Pediatric Oncology, Hematology and Clinical Immunology, University Children's Hospital, Heinrich Heine University, Düsseldorf, Germany.
Michaela
M
Kuhlen
Department of Pediatric Oncology, Hematology and Immunology, Heidelberg University Hospital, Heidelberg, Germany.
Andreas E
AE
Kulozik
Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany.
Britta
B
Lamottke
Kiel Paediatric Tumor Registry, Department of Paediatric Pathology, University of Kiel, Kiel, Germany.
Ivo
I
Leuschner
Institute of Human Genetics, University Hospital Essen, Essen, Germany.|Eye Oncogenetics Research Group, University Hospital Essen, Essen, Germany.
Dietmar R
DR
Lohmann
Department of Pediatric Hematology and Oncology, Justus Liebig University, Giessen, Germany.
Andrea
A
Meinhardt
Department of Pediatrics and Adolescent Medicine, University of Erlangen-Nuremberg, Erlangen, Germany.
Markus
M
Metzler
Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany.
Lüder H
LH
Meyer
Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University Medical Center, Aachen, Germany.
Olga
O
Moser
Department of Pediatric Oncology, Klinikum Kassel, Kassel, Germany.|Clinical Cooperation Group Osteosarcoma, Helmholtz Zentrum Munich, Neuherberg, Germany.|Pediatric Oncology Center, Technical University Munich, Munich, Germany.
Michaela
M
Nathrath
Faculty of Medicine, Division of Pediatric Hematology and Oncology Medical Center, Department of Pediatrics and Adolescent Medicine, University of Freiburg, Freiburg, Germany.
Charlotte M
CM
Niemeyer
Department of Surgery, Children's Hospital, Hannover Medical School, Hannover, Germany.
Rainer
R
Nustede
Department of Pediatric Oncology, Hematology and Immunology, Heidelberg University Hospital, Heidelberg, Germany.|Division of Pediatric Neuro-Oncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
Kristian W
KW
Pajtler
Department of Pediatric Hematology/Oncology, University Medical Center Mainz, Mainz, Germany.
Claudia
C
Paret
Pediatric Oncology and Hematology, Pediatrics III, University Hospital of Essen, Essen, Germany.
Mareike
M
Rasche
Pediatric Oncology and Hematology, Pediatrics III, University Hospital of Essen, Essen, Germany.
Dirk
D
Reinhardt
Institute of Medical Genetics and Applied Genomics, University of Tuebingen, Tuebingen, Germany.
Olaf
O
Rieß
Department of Pediatric Hematology/Oncology, University Medical Center Mainz, Mainz, Germany.
Alexandra
A
Russo
Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Stefan
S
Rutkowski
Department of Human Genetics, Hannover Medical School, Hannover, Germany.
Brigitte
B
Schlegelberger
Clinic of Pediatrics, Dortmund, Germany.
Dominik
D
Schneider
Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Reinhard
R
Schneppenheim
Department of Pediatrics, University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany.
Martin
M
Schrappe
Institute of Medical Genetics and Applied Genomics, University of Tuebingen, Tuebingen, Germany.
Christopher
C
Schroeder
Department of Pediatric Surgery, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University, Munich, Germany.
Dietrich
D
von Schweinitz
Department of Pediatric Hematology and Oncology, University of Cologne, Cologne, Germany.
Thorsten
T
Simon
Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart-Olgahospital, Stuttgart, Germany.
Monika
M
Sparber-Sauer
German Childhood Cancer Registry (GCCR), Institute for Medical Biostatistics, Epidemiology and Informatics (IMBEI), University Medical Center of the Johannes Gutenberg-University Mainz, Mainz, Germany.
Claudia
C
Spix
Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany.
Martin
M
Stanulla
Department of Human Genetics, Hannover Medical School, Hannover, Germany.
Doris
D
Steinemann
Faculty of Medicine, Division of Pediatric Hematology and Oncology Medical Center, Department of Pediatrics and Adolescent Medicine, University of Freiburg, Freiburg, Germany.
Brigitte
B
Strahm
Pediatric Oncology and Hematology, Pediatrics III, University Hospital of Essen, Essen, Germany.|Eye Oncogenetics Research Group, University Hospital Essen, Essen, Germany.
Petra
P
Temming
Department of Human Genetics, Hannover Medical School, Hannover, Germany.
Kathrin
K
Thomay
Department of Pediatrics, Division of Pediatric Hematology and Oncology, University Medical Center Goettingen, Goettingen, Germany.|Division of Pediatric Hematology and Oncology, University Hospital of Geneva, Geneva, Switzerland.
Andre O
AO
von Bueren
Pediatric Oncology, Otto von Guericke University Children's Hospital, Magdeburg, Germany.
Peter
P
Vorwerk
Department of Pediatric Oncology, Hematology and Immunology, Heidelberg University Hospital, Heidelberg, Germany.
Olaf
O
Witt
Faculty of Medicine, Division of Pediatric Hematology and Oncology Medical Center, Department of Pediatrics and Adolescent Medicine, University of Freiburg, Freiburg, Germany.
Marcin
M
Wlodarski
Department of Pediatric Hematology and Oncology, Justus Liebig University, Giessen, Germany.
Willy
W
Wössmann
Institute of Human Genetics, University Hospital Magdeburg, Otto-von-Guericke University, Magdeburg, Germany.
Martin
M
Zenker
Hospital for Children and Adolescents, Goethe-University Frankfurt, Frankfurt am Main, Germany.
Stefanie
S
Zimmermann
Department of Pediatric Oncology, Hematology and Immunology, Heidelberg University Hospital, Heidelberg, Germany.|Division of Pediatric Neuro-Oncology, German Cancer Research Center (DKFZ), Heidelberg, Germany.
Stefan M
SM
Pfister
Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany.
Christian P
CP
Kratz
D000293:Adolescent; D002648:Child; D017144:Focus Groups / Q000379:methods; D015870:Gene Expression; D005817:Genetic Counseling / Q000941:ethics; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing / Q000379:methods; D005826:Genetics, Medical / Q000266:history / Q000295:instrumentation / Q000379:methods; D019337:Hematologic Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D049674:History, 21st Century; D006801:Humans; D009154:Mutation; D009363:Neoplasm Proteins / Q000235:genetics*; D009369:Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D012955:Societies, Medical / Q000266:history; D013577:Syndrome
D009363:Neoplasm Proteins
genetic cancer predisposition; genetic predisposition testing; genetic screening; germline mutation; hereditary cancer syndrome