ACaseofSecondaryHypogonadism“HypogonadismDuetoPituicytomainanIdenticalTwin”H.H.Newnham&L.M.Rivera-WollNewEnglJMed359:2824,2008W.RosePresentationandHistory43-year-oldman(anidenticaltwin)presentswith2-yearhistoryoflethargy,weakness,headaches;several-yearhistoryofgraduallossofbodyhairanddeclininglibido.Leftorchidopexyat6yearsofage.PhysicalExamShowsfeaturesofhypopituitarismandhypogonadism,includingcentraladiposity,nipplepallor,proximalmusclewasting,preservationofscalphair,lossofbodyhair,gynecomastia,andrightandlefttesticularvolumesof12mland5ml,respectively(normalvolume,>15).Notecontrastinappearancebetweenhypogonadalpatient(PanelA,right)andunaffectedidenticaltwin(left).Newnham,H.H.&L.M.Rivera-Woll(2008),NewEnglJMed359:2824.Newnham,H.H.&L.M.Rivera-Woll(2008),NewEnglJMed359:2824.LaboratoryTestsTestosterone:1.5nmolperliter(normalrange,9.9to27.8).Follicle-stimulatinghormone:2.8IUperliter(normalrange,1.5to12.4)Luteinizinghormone:1.5IUperliter(normalrange,1.7to8.6).Serumprolactin,thyroidfunction(measuredbyfreethyroxineandthyroid-stimulatinghormone),cortisol:normal.Insulin-likegrowthfactor1level:mildlydecreased.Newnham,H.H.&L.M.Rivera-Woll(2008),NewEnglJMed359:2824.ImagingMagneticresonanceimagingofthepituitaryshowedalobulated,contrast-enhancingsuprasellarmass(16by29mm;seeninthecoronalviewinPanelB[arrow]andinthesagittalviewinPanelC[arrow]).Imagesoftheunaffectedtwinareshownforcomparison.Newnham,H.H.&L.M.Rivera-Woll(2008),NewEnglJMed359:2824.Newnham,H.H.&L.M.Rivera-Woll(2008),NewEnglJMed359:2824.Pituicytoma,MRINormal,MRIDiagnosisPituicytoma=pituitarycelltumor,confirmedbypathologicalanalysisoftissueobtainedfromneedlebiopsyandsubsequentsurgery.TreatmentTumorexcisionbycraniotomy.FollowupApartfromaseizure,patient'spostoperativerecoveryonpituitary-replacementtherapyhasbeenuneventful,withresolutionofhissymptoms.Newnham,H.H.&L.M.Rivera-Woll(2008),NewEnglJMed359:2824.CommentsPituitarytumorsusuallygrowslowly,don’tmetastasize,andcanbeeffectivelytreatedbysurgicalremoval.Pituitarytumorscanbesecretoryornon-secretory.Asecretorytumormakesoneormoreofthehormonesmadebyanormalpituitary,butmakesthehormonesinanuncontrolledway(i.e.toomuch).W.RoseDiscussionQuestionsWhyaretestessmallandtestosteronelow?WhyisLtestissmallerthanR?WhatdoFSH&LHdo,andwherearetheyproduced?Inamalewithanormalpituitary,howwouldFSHandLHlevelsrespondtolowtestosterone?Comparetothispatient.Whyarelevelsofprolactin,thyroidhormoneandTSH,cortisol,andIGF-1checkedandreported?(Whicharemadeinpituitary,whichareregulatedbypituitary?)Isthispituitarytumorsecretoryornon-secretory?Whydoyousayso?Suprasellar?W.RoseDiscussionQuestionsWhatsymptommightbeexpectedthatwasnotseen?Howwouldyoudoaneedlebiopsyorremovethetumor?Whatstructureswouldyoubeespeciallycarefultoavoid?Ifanteriorpituitary(adenohypophysis)werecompletelyremoved,whathormoneswouldhavetobemonitoredandreplacedexogenously?Ifposteriorpituitary(neurohypophysis)werealsoremoved,whathormoneswouldbeaddedtothelist?W.RoseW.RoseTew,JM,Jr,vanLoveren,HR,Keller,JT;AtlasofOperativeMicroneurosurgery,VolumeII;W.B.Saunders,2002,retreivedon2009-01-08fromhttp://www.mayfieldclinic.com/PE-surgpit.htm.CoronalsectionConventionaltransphenoidalapproach.Craniotomyandsubfrontalapproach.W.RoseAnimationofdifferentsurgicalapproachestopituitarytumorsavailableathttp://www.skullbaseinstitute.com/animations.htmEndoscopictransphenoidalapproachhttp://www.ent.uci.edu/endoscopic_pituitary_tumor.htm