Adrenocortical Carcinoma: Basic Science and Clinical Concepts PDF
Preview Adrenocortical Carcinoma: Basic Science and Clinical Concepts
Adrenocortical Carcinoma · Gary D. Hammer Tobias Else Editors Adrenocortical Carcinoma Basic Science and Clinical Concepts 123 Editors GaryD.Hammer,MD,Ph.D. TobiasElse,MD MilleSchembechlerProfessorofAdrenal DepartmentofInternalMedicine Cancer DivisionofMetabolism,Endocrinology Director–EndocrineOncologyProgram &Diabetes Director–CenterforOrganogenesis UniversityofMichiganHealthSystem UniversityofMichiganHealthSystem UniversityofMichigan UniversityofMichigan ISBN978-0-387-77235-6 e-ISBN978-0-387-77236-3 DOI10.1007/978-0-387-77236-3 SpringerNewYorkDordrechtHeidelbergLondon LibraryofCongressControlNumber:2010934103 ©SpringerScience+BusinessMedia,LLC2011 Allrightsreserved.Thisworkmaynotbetranslatedorcopiedinwholeorinpartwithoutthewritten permission of the publisher (Springer Science+Business Media, LLC, 233 Spring Street, New York, NY10013,USA),exceptforbriefexcerptsinconnectionwithreviewsorscholarlyanalysis.Usein connectionwithanyformofinformationstorageandretrieval,electronicadaptation,computersoftware, orbysimilarordissimilarmethodologynowknownorhereafterdevelopedisforbidden. Theuseinthispublicationoftradenames,trademarks,servicemarks,andsimilarterms,eveniftheyare notidentifiedassuch,isnottobetakenasanexpressionofopinionastowhetherornottheyaresubject toproprietaryrights. Whiletheadviceandinformationinthisbookarebelievedtobetrueandaccurateatthedateofgoing topress,neithertheauthorsnortheeditorsnorthepublishercanacceptanylegalresponsibilityforany errorsoromissionsthatmaybemade.Thepublishermakesnowarranty,expressorimplied,withrespect tothematerialcontainedherein. Printedonacid-freepaper SpringerispartofSpringerScience+BusinessMedia(www.springer.com) Preface Adrenocorticalcarcinoma(ACC)isadiseasethatmostphysicians,includingmany endocrinologists, will rarely, if ever, diagnose or let alone treat during the course oftheirmedicalpractice.Medicaltextbooksofendocrinologyandoncologyrarely dedicate an entire chapter to this disease entity. The pursuit of research and clini- calexcellenceinuncommondiseasesisextremelychallengingbecauseofalackof researchprioritization,nonexistenttreatmentguidelinesandoverallpaucityofcoor- dination between researchers and physicians. ACC is one such disease where no infrastructureforaunifiedresearchagendaandnoconsensustreatmentguidelines hadbeendeveloped. Whileanumberofinternationalmeetingsoverthepasttwodecadeshaveindeed focusedonadrenaltumorsinthecontextofhormoneexcess(Cushing’ssyndrome, hyperaldosteronism, and pheochromocytoma), few have exclusively catered to the science and clinical care of ACC and those afflicted with the disease. With only a single FDA-approved drug for ACC (mitotane – a derivative of the pesticide DDT), institutional experiences varied widely until recently when historic biases haveslowlyyieldedtodata-driventreatmentstrategies.Alargepartoftheimpetus for this push has come from Europe, where the availability of country-wide inte- gratednetworksfortreatmenthasallowedasmallnumberofcentersinItaly,France, and Germany (among others) to develop specific expertise and specific treatment protocolsforthisraredisease. Inanattempttofacilitatecoordinationofglobalefforts,aconsensusconference was organized and held at the University of Michigan in September 2003. At that meeting, an international group of physicians and scientists with research interest and clinical expertise in ACC set up initial guidelines for the diagnosis and treat- ment of ACC. Three principles emerged. Successful treatment of ACC demands coordinated care in the context of a multidisciplinary team dedicated to the dis- ease.FuturetherapiesforACCneedtobepredicatedonhypothesis-drivenresearch based on a thorough analysis of tumor biology. Lastly, major advancement in the field demands national and international collaborative networks to facilitate anal- ysis of large datasets and coordinate future clinical trials. The FIRM-ACT (First InternationalRandomizedTrialinlocallyadvancedandMetastaticAdrenocortical Cancer Treatment) that coordinated over 35 ACC centers in a single multina- tional trial set precedence for the actualization of these principles. The Second v vi Preface International Adrenal Cancer Symposium: Clinical and Basic Science held at the University of Michigan in March 2008 built upon the momentum of the 2003 consensus meeting and the successful development of a large international ACC networkthroughtheFIRM-ACTtrial. Overthepastdecade,theACCresearchcommunityhasgrowntoacriticalmass with new data emerging in the laboratory and clinic. In times of electronic pub- lications we routinely rely on journal articles and expert reviews on both clinical andresearchtopics.Whilesuchpublicationsareinformative,whenapproachedby Springeraboutthepossibilityofeditingsuchatextbook,webecameconvincedthat thetimehadcometocompiletheaccumulatedclinicalandbasicscienceknowledge of 50 years of active research on this rare cancer into a concise medical textbook. Theoverallgoalofthisbookisthereforetoprovidedefinitivereferencematerialfor scientistsandclinicians,tointroducetraineestoconceptsofACCmanagement,and tostimulatefurtherresearch,futurecollaborations,andnetworking. As opposed to a solitary review article, a textbook with multiple chapters dedicated to discrete topics in the field provides contributors the opportunity to objectively review historic data and detail the current state of clinical care and researchaccomplishments.Whilethisisamajoradvantageofatextbook,itisalsoa majorchallengeforabookthatfocusessolelyonararecancerwheredataarescant. In editing this book, we tried to ensure that each individual chapter covers well- establishedknowledgeinthearea,butalsoallowsroomforexpertopinion.Lastly, becauseACChasbeenlinkedtoseveralgeneticdisordersthatusuallyescapediscus- sioninafocusedreviewofadrenaltumors,thevarioussyndromeswillbediscussed intheirentiretyinseparatechapters.The32Chaptersofthe9Sectionsareauthored bythescientificandclinicalleadersinthefield. With publication of this first edition, the editors want to extend special thanks to our colleagues within the ACC community, the contributors, Rachel, Todd and LesleyoftheeditorialstaffatSpringerPublishingHouse,andLisaK.Byrdofthe UniversityofMichigan. Wearehopefulthatthisfirsteditionofthetextbookprovidesanintellectualplat- form for the continued coalescence and dissemination of knowledge on ACC in futureeditions.Boththeauthorsandeditorswelcomecommentsandrecommenda- tions for improvement in writing or via electronic mail. The editors’ and authors’ institutionalande-mailaddressesaregiveninthecontributor’ssection. AnnArbor,Michigan GaryHammer November2010 TobiasElse Contents PartI History of Adrenocortical Carcinoma Research andClinicalCare 1 TheHistoryofAdrenocorticalCarcinomaTreatment–A MedicalPerspective . . . . . . . . . . . . . . . . . . . . . . . . . . 3 DavidE.Schteingart 2 TheHistoryofAdrenocorticalCarcinomaTreatment–A SurgicalPerspective . . . . . . . . . . . . . . . . . . . . . . . . . . 9 NormanW.Thompson PartII Epidemiology,PresentationandDiagnosis 3 EpidemiologyofAdrenocorticalCarcinoma . . . . . . . . . . . . 23 MartinFassnachtandBrunoAllolio 4 ClinicalPresentationandInitialDiagnosis . . . . . . . . . . . . . 31 BrunoAllolioandMartinFassnacht 5 DiagnosticApproachtoIncidentaloma . . . . . . . . . . . . . . . 49 HolgerS.WillenbergandStefanR.Bornstein PartIII Imaging 6 ComputedTomography/MagneticResonanceImaging ofAdrenocorticalCarcinoma . . . . . . . . . . . . . . . . . . . . 67 MelvynKorobkin,AncaM.Avram,andHeroK.Hussain 7 FunctionalImagingofAdrenocorticalCarcinoma . . . . . . . . . 85 AncaM.AvramandStephanieHahner PartIV Pathology 8 ClassicalHistopathologyandImmunohistochemistry . . . . . . . 107 WolfgangSaeger vii viii Contents 9 Cellular and Molecular Pathology of Adrenocortical Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 TobiasElse PartV GeneticandMolecularAspects 10 Overview of Genetic Syndromes Associated withAdrenocorticalCancer . . . . . . . . . . . . . . . . . . . . . 153 TobiasElse 11 Li–FraumeniSyndrome . . . . . . . . . . . . . . . . . . . . . . . 173 DavidMalkin 12 TP53MolecularGenetics . . . . . . . . . . . . . . . . . . . . . . . 193 GerardP.ZambettiandRaulC.Ribeiro 13 TelomeresandTelomeraseinAdrenocorticalCarcinoma . . . . . 207 TobiasElseandPeterJ.Hornsby 14 Beckwith–WiedemannSyndrome . . . . . . . . . . . . . . . . . . 227 MichaelDeBaunandJenniferHorst 15 TheInsulin-LikeGrowthFactorSysteminAdrenocortical GrowthControlandCarcinogenesis . . . . . . . . . . . . . . . . . 235 ChristianFottner,InaM.Niederle,andMatthiasM.Weber 16 WNT/β-CateninSignalinginAdrenocorticalCarcinoma . . . . . 263 SébastienGaujoux,FrédériqueTissier,andJérômeBertherat PartVI ModelsofAdrenocorticalCancer 17 AdrenocorticalStemandProgenitorCells: ImplicationsforCancer . . . . . . . . . . . . . . . . . . . . . . . . 285 JoanneH.HeatonandGaryD.Hammer 18 AdrenocorticalCellLines. . . . . . . . . . . . . . . . . . . . . . . 305 JenielParmar,AnitaKulharya,andWilliamRainey 19 MouseModelsofAdrenalTumorigenesis . . . . . . . . . . . . . . 325 FelixBeuschlein PartVII Therapies 20 OverviewofTreatmentOptionsforAdrenocorticalCarcinoma. . 343 GaryD.Hammer 21 Chemotherapy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351 AlfredoBerruti,PaolaSperone,PaolaPerotti,AnnaFerrero, LuigiDogliotti,andMassimoTerzolo 22 Mitotane . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369 MassimoTerzolo,AriannaArdito,BarbaraZaggia,SilviaDe Francia,andFulviaDaffara Contents ix 23 PharmacotherapyforHormoneExcessinAdrenocortical Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 383 RichardJ.Auchus 24 SurgeryforAdrenocorticalCarcinoma . . . . . . . . . . . . . . . 403 JamesT.Broome,BarbraS.Miller,PaulG.Gauger, andGerardM.Doherty 25 RadiationTherapyforAdrenocorticalCarcinoma . . . . . . . . . 427 AaronSabolchandEdgarBen-Josef 26 Follow-UpandMonitoringofAdrenocorticalCarcinoma . . . . . 443 BrittSkogseidandGerardM.Doherty PartVIII UniqueCohortsandFuturePerspectives 27 Aldosterone-ProducingAdrenocorticalCarcinoma . . . . . . . . 457 AnnaPatalano,MariaV.Cicala,andFrancoMantero 28 AdrenocorticalCancerinChildren . . . . . . . . . . . . . . . . . 467 CarlosRodriguez-Galindo,GerardP.Zambetti, andRaulC.Ribeiro 29 Genome-WideStudiesinAdrenocorticalNeoplasia . . . . . . . . 483 ThomasJ.Giordano 30 New Strategies for the Treatment of Adrenocortical Carcinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493 LawrenceS.Kirschner PartIX AdrenalCancerNetworksandRegistries 31 TheDutchAdrenalNetwork . . . . . . . . . . . . . . . . . . . . . 517 IlseG.C.Hermsen,YvonneE.Groenen,andHarmR.Haak 32 TheENS@TInitiative . . . . . . . . . . . . . . . . . . . . . . . . 521 XavierBertagna Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 533 Contributors BrunoAllolio DepartmentofInternalMedicineI,EndocrineandDiabetesUnit, UniversityofWürzburg,Josef-Schneider-Str.2,97080Würzburg,Germany, [email protected] AriannaArdito DepartmentofClinicalandBiologicalSciences,Medicina Interna1,AOUSanLuigiGonzaga,UniversityofTurin,RegioneGonzole,10, 10043Orbassano,Italy,[email protected] RichardJ.Auchus DivisionofEndocrinologyandMetabolism,Departmentof InternalMedicine,UTSouthwesternMedicalCenterDallas,5323HarryHines Boulevard,Dallas,TX75390,USA,[email protected] AncaM.Avram DivisionofNuclearMedicine/Radiology,Universityof MichiganHealthSystem,UniversityofMichigan,1500EastMedicalCenter Drive,AnnArbor,MI48105,USA,[email protected] EdgarBen-Josef DepartmentofRadiationOncology,UniversityofMichigan HealthSystem,UniversityofMichigan,1500EastMedicalCenterDrive,Room UHB2C490,AnnArbor,MI48109-0010,USA,[email protected] AlfredoBerruti OncologiaMedica,AziendaOspedalieroUniversitariaSanLuigi, RegioneGonzole,10,10043Orbassano,Italy,[email protected] JérômeBertherat Endocrinology,MetabolismandCancerDepartment,Institut Cochin,DescartesUniversity,INSERMU567,CNRSUMR8104,ParisFrance; Referencecenterforrareadrenaldisorders,AssistancePubliqueHôpitauxdeParis, HôpitalCochin,ParisDescartesUniversity,27RueduFaubourgSaint-Jacques, 75014Paris,France,[email protected] XavierBertagna EndocrinologyDepartment,CochinHospital,Paris,France; NationalNetworkCOMETE,INCa,Paris,France;EuropeanNetworkforthe Studyof@drenalTumors(ENS@T),27RueduFaubourgSaint-Jacques,75014 Paris,France,[email protected] FelixBeuschlein DepartmentofMedicine,EndocrineResearch,University HospitalInnenstadt,Ziemssenstr.1,80336Munich,Germany, [email protected] xi
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