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38 th Congress of the International Society of Paediatric Oncology Geneva, Switzerland · September 17-21, 2006 1 -2 71 e G neva Se em er pt b SIOP EDUCA SIOP EDUCA SIOP EDUCA SIOP EDUCA SIOP EDUCATION BOOK 2006 TION BOOK 2006 TION BOOK 2006 TION BOOK 2006 TION BOOK 2006 International Society of Paediatric Oncology www.siop.nl

International Society of Paediatric Oncology

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38th Congress of the International Society of Paediatric Oncology

Geneva, Switzerland · September 17-21, 2006

1 -27 1eG neva

Se em erpt b

SIOP EDUCASIOP EDUCASIOP EDUCASIOP EDUCASIOP EDUCATION BOOK 2006TION BOOK 2006TION BOOK 2006TION BOOK 2006TION BOOK 2006

International Society ofPaediatric Oncology

www.siop.nl

SIOP EDUCASIOP EDUCASIOP EDUCASIOP EDUCASIOP EDUCATION BOOK 2006TION BOOK 2006TION BOOK 2006TION BOOK 2006TION BOOK 2006

International Society ofPaediatric Oncology

38th Congress of the International Society of Paediatric OncologyGeneva, Switzerland · September 17-21, 2006

www.siop.nl

2

3

S I O PInternational Societyof Paediatric Oncology

E D I T O R S

Bharat R. AgarwalChair, SIOP EducationCommittee & Secretary General,SIOP

Giorgio PerilongoChair, Scientific CommitteeSIOP

Pierre WackerChair, Local OrganizingCommittee39th Congress of SIOP

Tim EdenPresident, SIOP

A U T H O R S

Section A

Educational Session: Genetics andpharmacogenetics in pediatric leukaemia

Andrea Biondi Monique den Boer

Tim Eden Pamela Kearns

Max Van Noesel Rob Pieters

Ching-Hon Pui Kjeld Schmiegelow

Micheal Zwaan

Section B

Keynote Lectures

Maurizio Arico Stefan Bielack

Sarah Donaldson Mhamed Harif

Fred Hoffer Stefan Meyer

Tom Monclair Paul Rogers

Paul Scotting Kevin Shannon

Mary Dixon Woods

State of the Art Lectures

Henrik Hasle Giorgio Perilongo

Jean Bernard Otte Lillian Sung

Derek Roebuck

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© 2006 International Society of Paediatric Oncology. All rights reserved. No parts

of this publication may be reproduced, stored in a retrieval system, or transmitted

in any form or by any means, electronic or mechanical, including photocopy,

without prior consent of the International Society of Paediatric Oncology.

First published 2006

For further information

Visit our website: http://www.siop.nl

A c k n o w l e d g e m e n t s

The International Society of Paediatric Oncology would like to acknowledge the

efforts of the authors. SIOP is immensely grateful to all the authors for not only

presenting state of the art lectures and occasional sessions at the meeting

but for agreeing to produce a manuscript to assist in ongoing education for

participants at the 38th meeting of SIOP in Geneva 2006.

D i s c l a i m e r

The contents of this book represent the views of the individual authors and not

necessarily of SIOP.

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S I O PInternational Societyof Paediatric Oncology

C O N T E N T S

Authors ............................................................................................................. 7

Preface ............................................................................................................. 9

Section A - Educational Session :Genetics and pharmacogenetics in pediatric leukemia

1. Steroids in ALLTim Eden .................................................................................................... 12

2. How to adapt drug doses in maintenance therapy of ALLKjeld Schmiegelow .................................................................................... 17

3. Pharmacogenetics and drug dosing in children with Down syndromeMicheal Zwaan ........................................................................................... 29

4. Pharmacogenetics in childhood Acute Lymphoblastic LeukemiaChing-Hon Pui ............................................................................................ 36

5. From gene expression profiles data to new targeted therapies in ALLMonique den Boer ..................................................................................... 39

6. Epigenetic Alterations and targeted chromatin remodeling drugs inpediatric leukemiaMax Van Noesel ......................................................................................... 42

7. Treatment of MLL gene rearranged ALLRob Pieters ................................................................................................ 48

8. Treatment of Philadelphia positive (Ph+) ALL and CML in childrenAndrea Biondi ............................................................................................ 55

9. New molecular targets for therapy in leukemiaPamela Kearns ........................................................................................... 59

Section B – Keynote lectures and “State of the Art” Lectures

1. The new international neuroblastoma Risk Group Staging System -Implications for surgeonsTom Monclair ............................................................................................. 64

2. The relevance of nutrition to pediatric oncologyPaul Rogers ............................................................................................... 67

3. The expanded future of pediatric radiation oncologySarah Donaldson ....................................................................................... 71

4. Hemophagocytic lymphohistiocytosis- A challenge for the pediatrichematologist-oncologistMaurizio Arico ............................................................................................ 74

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5. Understanding experiences of childhood cancer: a fresh perspectiveMary Dixon Woods ..................................................................................... 77

6. Pediatric Hematology/Oncology in MoroccoMhamed Harif ............................................................................................ 80

7. Childhood brain tumours: a developmental disorderPaul Scotting .............................................................................................. 83

8. Understanding cellular pathways of DNA damage recognition andrepair - Clinical implicationsStefan Meyer .............................................................................................. 88

9. Pathogenic mechanisms in Juvenile myelomonocytic leukaemiaKevin Shannon ........................................................................................... 92

10. Minimally invasive diagnosis and treatment of pediatric malignanciesthrough interventional radiologyFred Hoffer ................................................................................................. 96

11. OsteosarcomaStefan Bielack ........................................................................................... 99

“State of the Art” Lectures

1. Preventing Invasive Bacterial Infection in Neutropenic Patients withCancerLillian Sung .............................................................................................. 113

2. Radiological staging of primary malignant liver tumors in childrenDerek Roebuck ........................................................................................ 119

3. Treatment of hepatoblastomaGirogio Perilongo, Derek Roebuck, Jean Bernard Otte .......................... 122

4. Treatment of hepatoblastoma - Role of surgeryJean Bernard Otte .................................................................................... 128

5. Myelodysplastic Syndrome (MDS) and juvenline myelomonocyticleukaemia (JMML)Henrik Hasle ............................................................................................ 130

C O N T E N T S

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S I O PInternational Societyof Paediatric Oncology

Editors

Bharat AgarwalHead, Dept. of PediatricHematology-Oncology,B.J.Wadia Hospital forChildren, Parel,[email protected]

Giorgio PerilongoClinicia Pediatrica 2University Of PadovaVia Giustiniani 335128 [email protected]

Pierre WackerPediatric Hematology-Oncology Unit ; HUG6, Rue Willy Donze1211 Geneva [email protected]

Tim EdenProfessor of Adolescent,Teenage and Young AdultCancerYoung Oncology UnitUniversity of [email protected]

Section A

Tim EdenProfessor of Adolescent,Teenage and Young AdultCancerYoung Oncology UnitUniversity of [email protected]

Kjeld SchmiegelowDr. Med. Sci., professor,The Paediatric Clinic,The University Hospital ofCopenhagen,Rigshospitalet, [email protected]

Micheal Zwaan,Dept. of Pediatric Oncology/HematologyErasmus MC/SophiaChildren’s HospitalRotterdamThe [email protected]

Ching-Hon PuiSt. Jude Children’s ResearchHospital, andUniversity of TennesseeHealth Science Center,Memphis, TN [email protected]

Monique L. den BoerErasmus MC-SophiaChildrens HospitalDept of Oncology/HematologyRoom Sp2456Dr Molewaterplein 603015GJ RotterdamThe [email protected]

Max M. van NoeselDepartment of pediatriconcology/hematologyErasmus MC - SophiaChildren’s HospitalDr Molewaterplein 603015 GJ Rotterdam, [email protected]

Rob PietersErasmus MC SophiaChildrens HospitalDr Molewaterplein 603015GJ Rotterdamthe Netherlandstel + 31 10 463 6691fax + 31 10 463 [email protected]

Andrea BiondiOspedale San GerardoUniversita Milano BicoccaClinica PaediatricaVia Donizetti 106Monza [email protected]

Pamela KearnsDepts. of Clinical Sciencesand Cellular and MolecularMedicine,University of Bristol, [email protected]

E D I T O R S & A U T H O R S

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Section B

Tom MonclairRikshospitalet-Radiumhospitalet,The National UniversityHospital,Oslo, [email protected]

Paul Rogers4480 Oak StreetV6H 3V4 [email protected]

Sarah DonaldsonDepartment of RadiationOncology,Stanford University,Stanford,California, 94305, [email protected]

Maurizio AricoPediatric Hematology-Oncology,Ospedale dei Bambini“G. Di Cristina”,Palermo,[email protected]

Mary Woods DixonReader in Social Science andHealth,Department of HealthSciences,University of Leicester,[email protected]

Mhamed HarifService d’Hématologie etOncologie Pédiatrique, Hôpital 20 août 1953,Casablanca, Morocco,[email protected]

Paul ScottingChildren’s Brain TumourResearch Centre.Institute of GeneticsUniversity of Nottingham,Queen’s Medical Centre,Nottingham, [email protected]

Stefan MeyerPaediatric Oncology,University of Manchester,[email protected]

Kevin ShannonDepartment of Pediatrics andComprehensive CancerCenter,University of California,San Francisco,CA 94143 [email protected]

Fredric A. HofferPediatric and InterventionalRadiologistDivision of DiagnosticImagingDepartment of RadiologicalSciencesMember & Chair of theClinical Staff St. Jude Children’s ResearchHospital332 N. Lauderdale St.,Memphis, TN 38105 [email protected]

Stefan BielackOlgahospitalPediatrics 5(Oncology, Hematology,Immunology)Bismarckstr. 8D-70176 [email protected]

Lillian SungHospital for Sick ChildrenToronto, [email protected]

Derek RoebuckDepartment of RadiologyGreat Ormond Street HospitalLondon WC1N 3JHUnited [email protected]

Giorgio PerilongoClinicia Pediatrica 2University Of PadovaVia Giustiniani 335128 PADOVA, [email protected]

Jean-Bernard Otte,Professor emeritus at theUniversitéCatholique de LouvainFormer head of the LiverTransplantationProgram and the Departmentof Pediatric SurgeryCliniques Universitaires Saint-Luc, Brussels, [email protected]

Henrik HasleDepartment of PediatricsSkejby Hospital, Aarhus,[email protected]

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S I O PInternational Societyof Paediatric Oncology

P r e f a c e

At each SIOP meeting we attempt to bring together many of those who are working

in the field of paediatric haematology and oncology worldwide to share our

experiences and our expertise. SIOP has gradually developed in recent years an

increasing educational component to the meeting including specific pre-meeting

educational sessions and a series of keynote lectures and state of the art talks.

In 2005 we put those talks together in an educational book which we have tried to

make available to those who obviously attend the meeting but also worldwide to

members and those who have access to the website. I am most grateful to those

who agreed to talk and present their papers that they are willing to contribute to

this important educational document. We hope that those who can attend the

lectures and those who can’t but are able to read this book find it useful and of

course educational. The book demonstrates the wide breadth of content of current

SIOP meetings. It is a good advertisement for the annual meeting. If you are

reading this book and are not a member you can see why you should become

one.

Enjoy the book and the talks..

Tim Eden Giorgio PerilongoPresident, SIOP Chair, Scientific Committee,

SIOP

Bharat Agarwal Pierre WackerChair, Local Organizing CommitteeSIOP Education Committee & 38th Congress of SIOPSecretary General, SIOP