S P E URGICAL ATHOLOGY OF NDOCRINE AND N T EUROENDOCRINE UMORS C C P URRENT LINICAL ATHOLOGY ANTONIO GIORDANO, MD, PHD SERIES EDITOR Forfurthertitlespublishedinthisseries,goto http://www.springer.com/springer/series/7632 S P URGICAL ATHOLOGY E OF NDOCRINE AND N T EUROENDOCRINE UMORS Edited by A K SHRAF HAN, MD, FRCPath University of Massachusetts Medical School, Worcester, MA, USA Editor AshrafKhan DepartmentPathology UniversityofMassachusettsMedicalSchool(UMMS) UMassMemorialMedicalCenter 55LakeAvenueN. WorcesterMA01655 USA ISBN978-1-60327-395-4 e-ISBN978-1-60327-396-1 DOI10.1007/978-1-60327-396-1 LibraryofCongressControlNumber:2009928457 #HumanaPress,apartofSpringerScienceþBusinessMedia,LLC2009 Allrightsreserved.Thisworkmaynotbetranslatedorcopiedinwholeorinpartwithoutthewrittenpermissionofthepublisher(HumanaPress,c/o SpringerScienceþBusinessMedia,LLC,233SpringStreet,NewYork,NY10013,USA),exceptforbriefexcerptsinconnectionwithreviewsor scholarlyanalysis.Useinconnectionwithanyformofinformationstorageandretrieval,electronicadaptation,computersoftware,orbysimilaror dissimilarmethodologynowknownorhereafterdevelopedisforbidden. Theuseinthispublicationoftradenames,trademarks,servicemarks,andsimilarterms,eveniftheyarenotidentifiedassuch,isnottobetakenasan expressionofopinionastowhetherornottheyaresubjecttoproprietaryrights. Whiletheadviceandinformationinthisbookarebelievedtobetrueandaccurateatthedateofgoingtopress,neithertheauthorsnortheeditorsnor thepublishercanacceptanylegalresponsibilityforanyerrorsoromissionsthatmaybemade.Thepublishermakesnowarranty,expressorimplied, withrespecttothematerialcontainedherein. Printedonacid-freepaper SpringerispartofSpringerScienceþBusinessMedia(www.springer.com) To Rabab (Neelo) my loving wife, a great friend, the most caring person in the world and our two beautiful children Zehra and Faraan P REFACE Surgicalpathologyisthecornerstoneinmanagementofneoplasticdisorders.Withtheadvancesmadein our understanding of disease and application of new ancillary diagnostic studies the cross talk between surgical pathology and its related fields such as cytopathology and molecular diagnosis has become important for patient care and to provide the most accurate diagnosis. Endocrine tumors are common surgicalspecimensthatpathologistsencounterintheirpractice,sothetextpresentedhereshouldbeuseful formostgeneralpathologistsandpathologistsintraining. In addition to the tumors of the usual endocrine glands we have included endocrine tumors of non- endocrine organs as well. As is discussed later by Dr. Runjan Chetty this system of specialized peptide producingcellsthataredistributedinvariousorgansthroughoutthebodysharemanypropertieswithnerve cells;soforthisgroupoftumorstheterminology‘‘neuroendocrinetumors’’ispreferredinthisbook.While theemphasisinthisbookisonthesurgicalpathologyofendocrineandneuroendocrinetumorswehavealso provided sections on radiological imaging, cytologic diagnosis, and application of molecular diagnosis in these tumors. While the scope of the book does not allow us to go into the details, clinically pertinent informationrelevantforasurgicalpathologistisprovidedinthesesectionsandwehopethereaderswillfind thisinformationusefulinbetterunderstandingthesedisorders. The book was conceptualized with two main aims, one to put together an up-to-date text on surgical pathologyofendocrineandneuroendocrinetumorsthatispracticalkeepingthepracticingsurgicalpathol- ogistinmindandsecond,tolayoutthesectionsinsomewaysreminiscenttohowthediseasepresentsand thecoursetakenduringdiagnosis.Wethereforestartwithradiologicalimagingoftumors,whichmaybeone ofthefirstinvestigationsapatientundergoesfollowedbyasectiononfine-needleaspirationbiopsythatis the most important modality for pre-operative diagnosis that greatly assists in the surgical management. Thisleadsintoourmainsectiononsurgicalpathologyofendocrinetumors,whichisthefocusofthebook andfinallyweendwithapplicationofmoleculartechniques,whicharebecomingmoreandmoreimportant and have great potential for the future. The authors selected to contribute to this book are a group of internationally recognized pathologists who are not only experts and have extensively published in their respectivefieldsbutmoreimportantlyareexperiencedpracticingpathologistswhobringtheirownvaluable insightswhendiscussingthesetumors.Iampersonallyverygratefultoallthecontributorswhohavetaken time out of their very busy schedules to write these chapters. I would also like to take this opportunity to thanktheeditorialstaffatHumana/Springerforalltheirassistanceandmakingthisprojectpossible. WorcesterMA AshrafKhan vii C ONTENTS ImagingofEndocrineandNeuroendocrineTumors ................................................................................ 1 GulMoonisandKalpanaMani FineNeedleAspirationCytologyofEndocrineTumors .......................................................................... 9 SanjayLoganiandZubairW.Baloch FineNeedleAspirationCytologyofNeuroendocrineTumorsArisinginNon-endocrineOrgans ......... 19 SanjayLoganiandZubairW.Baloch TumorsofthePituitaryGland ............................................................................................................... 27 RicardoV.Lloyd,BerndW.Scheithauer,EvaHorvath,andKalmanKovacs TumorsofThyroidGland:Non-CcellTumors ...................................................................................... 41 AshrafKhanandManjuPrasad TumorsoftheThyroidGland(C-Cells) ................................................................................................. 83 RonaldA.DeLellis TumorsofParathyroidGland ................................................................................................................ 99 ManjuL.PrasadandAshrafKhan TumorsoftheAdrenalCortex ............................................................................................................. 111 AnneMarieMcNicol TumorsoftheAdrenalMedullaandExtra-adrenalParaganglia .......................................................... 121 ArthurS.TischlerandRonaldR.deKrijger EndocrineTumorsoftheLungandUpperAirways ............................................................................ 131 ArmandoE.Fraire,UlrikeM.Gruber-Mo¨senbacher,andHelmutH.Popper NeuroendocrineTumorsofthePancreas ............................................................................................. 143 RunjanChetty NeuroendocrineTumorsoftheGastrointestinalTract ......................................................................... 155 RunjanChetty NeuroendocrineTumoursoftheBreast ............................................................................................... 165 AndrewM.HanbyandRebeccaA.Brannan NeuroendocrineTumorsofFemaleGenitalTract ............................................................................... 173 KhushMittalandFanChen NeuroendocrineTumorsofProstate,UrinaryBladderandKidney ..................................................... 183 ZhongJiang NeuroendocrineTumorsofOtherMiscellaneousSites:ThymusandSkin ........................................... 191 FrancoiseTruongandAshrafKhan EndocrineTumorsandTumor-LikeLesionsofInfancy,Childhood,andAdolescentsand InheritedTumorSyndromes ................................................................................................................. 201 VaˆniaNose´ ix x Contents ApplicationofMolecularDiagnosisTechniquesintheDiagnosisandManagement ofEndocrineTumors ............................................................................................................................ 221 JenniferL.Hunt Index ..................................................................................................................................................... 235 C ONTRIBUTORS ZUBAIR W. BALOCH, MD, PHD (cid:2) Professor of Pathology, Director, Cytopathology Fellowship Program, Department of Pathology and Laboratory Medicine, University of Pennsylvania Medical Center, Philadelphia, PA 19104, USA, [email protected] REBECCA A. BRANNAN (cid:2) FRCPath MSc MBChB, Department of Histopathology, St James’s University Hospital, Beckett Street,Leeds,LS97TF,UnitedKingdom,[email protected] FANCHEN,MD,MD,PHD (cid:2) ClinicalAssistantProfessor,NYUSchoolofMedicineandHospitals,DepartmentofSurgical andOb-Gynpathology,BellevueHospital-BuildingH,Room4west,NY10016,USA,[email protected] RUNJAN CHETTY, MD, FRCPATH (cid:2) Professor of Pathology, Director of Surgical Pathology, Health Network/Toronto MedicalLaboratories,DepartmentofLaboratoryMedicineandPathobiology,UniversityofToronto,Toronto,Canada, [email protected] RONALD R. DE KRIJGER, MD (cid:2) Department of Pathology, Erasmus MC, 3000 DR Rotterdam, The Netherlands, [email protected] RONALDA.DELELLIS,M.D. (cid:2) Pathologist-in-Chief,LifespanAMCPathologyLaboratories,ProfessorandAssociateChair of Pathology and Laboratory Medicine, The Warren Alpert Medical School of Brown University, Department of Pathology,RhodeIslandHospital,Providence,RI02903,USA,[email protected] ANDREWM.HANBY,MD,FRCPATH (cid:2) Leeds InstituteofMolecularMedicine,YorkshireCancerResearchandLizDawn Pathology and Translational Sciences Centre Section of Pathology and Tumour Biology Wellcome Trust Brenner Building, Level 4, Room 4.13 St James’s University Hospital, Beckett Street Leeds, LS9 7TF, United Kingdom, [email protected] EVA HORVATH, PHD (cid:2) Department of Pathology, St Michael’s Hospital, Tronto, Ontario, CA, USA, [email protected] ARMANDOE.FRAIRE,MD (cid:2) ProfessorofPathology,Director,PulmonaryandAutopsyPathology,UniversityofMassachu- setts Medical School, UMass Memorial Medical Center, Three Biotech, One Innovation Drive, Worcester, MA, USA, [email protected] JENNIFER L. HUNT,M.D (cid:2) AssociateChiefof Pathology,DirectorofQualityand Safety,JamesHomer WrightPathology Laboratories,WRN225,MassachusettsGeneralHospital,55FruitStreet,Boston,MA02114,[email protected] ZHONG JIANG, MD (cid:2) Professor of Pathology, Director, Genitourinary Pathology, University of Massachusetts Medical School,ThreeBiotech,OneInnovationDriveWorcester,MA,USA,[email protected] ASHRAFKHAN,MD,FRCPATH (cid:2) ProfessorofPathology,Director,SurgicalPathology,DepartmentofPathology,University of Massachusetts Medical School, UMass Memorial Medical Center, Three Biotech, One Innovation Drive, Worcester, MA01605,USAe-mail:[email protected] KALMAN KOVACS, MD, PHD (cid:2) Department of Pathology, St Michael’s Hospital, Toronto, Ontario, CA, USA, [email protected] RICARDO V. LLOYD, M.D., PH.D (cid:2) Professor of Pathology and Laboratory Medicine, Division of Anatomic Pathology, MayoClinic,Rochester,MN55905,USA,[email protected] SANJAY LOGANI, MD (cid:2) Associate Professor of Pathology, Emory University School of Medicine, Department of Pathology, SuiteH187,1364CliftonRd,NE,Atlanta,GA30322,USA,[email protected] KALPANAMANI,MD (cid:2) DepartmentofRadiology,BethIsraelDeaconessMedicalCenter,HarvardMedicalSchool,Boston, MA,USA,[email protected] ANNEMARIEMCNICOL,MD,FRCPATH (cid:2) ProfessorMolecularandCellularPathology,SchoolofMedicine,Universityof Queensland,Brisbane,Australia,[email protected] KHUSH MITTAL, MD (cid:2) Associate Professor of Pathology, NYU School of Medicine and Hospitals, Director, Surgical and Ob-Gyn pathology, Bellevue Hospital – Building H, Room 4west, NY 10016, USA, [email protected] xi xii Contributors GULMOONIS,MD (cid:2) AssistantprofessorofRadiology,DivisionofNeuroradiology,BethIsraelDeaconessMedicalCenter, HarvardMedicalSchool,Boston,MA,USA,[email protected] VAˆNIANOSE´,MD,PH.D (cid:2) AssociateDirectorofSurgicalPathology,Chief,EndocrinePathologyService,AssociateProfessor ofPathology,DepartmentofPathology,HarvardMedicalSchool,BrighamandWomen’sHospital,Boston,MA02115, USA,[email protected] ULRIKEM.GRUBER-MOESENBACHER,MD (cid:2) InstituteofPathology,TeachingHospitalFeldkirchCarinagasse47,A-6800 Feldkirch,Austria,[email protected] HELMUTH.POPPER,MD,PROF.PATH (cid:2) InstituteofPathology,ResearchUnitMolecularLungandPleuraPathology,Lab. Molecular Genetics, Environmental und Respiratory Pathology, Medical University of Graz, Auenbruggerplatz 25,A-8036,Graz,Austria,[email protected] MANJUL.PRASAD,MD (cid:2) AssociateProfessor,Director,Endocrine,Head&NeckPathology,Director,Immunohistochem- istryLaboratory,DepartmentofPathology,YaleUniversitySchoolofMedicine,20YorkStreet,EP2-608B,NewHaven, CT06510,USA,[email protected] BERND W. SCHEITHAUER, MD (cid:2) Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First St SW, RochesterMN55905,USA,[email protected] ARTHUR S. TISCHLER, MD (cid:2) Professor of Pathology, Department of Pathology, Tufts University Tufts Medical Center, Boston,MA,[email protected] FRANCOISE TRUONG, MD FRCPC (cid:2) Consultant Pathologist, William Osler Health Center, 101 Humber College Blvd, Etobicoke,ON,Canada,M9VIR8,[email protected]