Primary lymphoma of the distal radius of a child: imaging features

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Case

Report

Primary

lymphoma

of

the

distal

radius

of

a

child:

imaging

features

Anna

Del

Poggio,

MD

_,

_Luca

_Facchetti,

_MD

_,

_Alessandra

_Ranza,

_MD

_,

_Fabio

_Facchetti,

_MD

PhD

_,

_Ugo

_Pazzaglia,

_MD

_,

_Maria

_Pia

_Bondioni,

_MD

a_{DepartmentofMedicalandSurgicalSpecialties,RadiologicalSciencesandPublicHealth,PediatricRadiology,} UniversityofBrescia,Brescia,Italy

b_{DepartmentofPediatricRadiology,ASSTSpedaliCivili,Brescia,Italy}

c_{DepartmentofMolecularandTranslationalMedicine,SectionofPathology,UniversityofBrescia,SpedaliCivili,} Brescia,Italy

a r t i c l e

i n f

o

a b s

t r a c t

Primarylymphomaofbone(PLB)isarareentity,definedasalymphomaconfinedtothe bonewithout evidenceofsystemicinvolvement.Thediseasecommonlyaffects middle-agedtoelderlypopulationanditaccountsforlessthan1%ofallmalignantlymphomas.We presentacaseofa10-year-oldchildaffectedbyPLBoftheforearmandthefrontalbone. CharacteristicimagingfeaturesofPLBandthemaindifferentialdiagnosiswerediscussed. © 2018TheAuthors.PublishedbyElsevierInc.onbehalfofUniversityofWashington.

ThisisanopenaccessarticleundertheCCBY-NC-NDlicense. (http://creativecommons.org/licenses/by-nc-nd/4.0/)

Introduction

Primarylymphomaofthebone(PLB)isararevariantof extra-nodalnon-Hodgkinlymphoma [1–3].AccordingtotheWorld HealthOrganizationclassification,lymphomainvolvingbone canbedividedintofourgroups:(1)singleskeletalsitewithout regionallymphnodeinvolvement;(2)multiple-bone

involve-Conflictofinterest:Theauthorsdeclarethattheyhavenoconflictofinterest. Funding:Thestudywasnotfunded.

Informedconsent:Informedconsentforpublicationofthiscasereportwasobtainedfromthepatient.

Ethicalapproval:Allproceduresperformedinthiscasereportwereinaccordancewiththeethicalstandardsoftheinstitutionaland/or nationalresearchcommitteeandwiththe1964Helsinkideclarationanditslateramendmentsorcomparableethicalstandards.

∗ _{Correspondingauthor.}

E-mailaddresses: delpoggio.anna@hsr.it(A.D.Poggio),l.facchetti007@unibs.it(L.Facchetti),radiologia.pediatrica@asst-spedalicivili.it

(A.Ranza),fabio.facchetti@unibs.it(F.Facchetti),ugo.pazzaglia@unibs.it(U.Pazzaglia),maria.bondioni@unibs.it(M.P.Bondioni).

mentwithoutvisceralorlymphnodeinvolvement;(3)bone lesionwithinvolvementofvisceralsitesor multiplelymph nodesatmultiplesites;and(4)patientwithknownlymphoma and bonebiopsy confirminginvolvementofbone.Groups1 and2areconsideredprimarylymphomasofthebone [3,4]. PLBaccountforlessthan1%ofallmalignantlymphomasand 7%ofmalignantbonetumors.Only4%-5%ofextranodal non-Hodgkin’slymphomasmanifestasPLB[2,5]andhistologically mostprimarybonelymphomasareprimarybonediffuselarge

https://doi.org/10.1016/j.radcr.2018.08.025

1930-0433/© 2018TheAuthors.PublishedbyElsevierInc.onbehalfofUniversityofWashington.Thisisanopenaccessarticleunderthe CCBY-NC-NDlicense.(http://creativecommons.org/licenses/by-nc-nd/4.0/)

B-celllymphomas(PBDLBCLs).Thediseasecommonlyaffects middle-aged to elderly population, with a median age of 48years [2].Inthisreport,wedescribethecaseofa 10-year-oldboyaffectedwithPLBpresentingwithararedouble in-volvementoftheforearmandthefrontalbone,focusingon PLBimagingfeaturesanddifferentialdiagnosis.

Case

report

A10-year-oldboycametoourattentionwitha2-month his-toryof pain inthe left forearm,in absence oftrauma. On physicalexamination,asubcutaneousswelling wasnotein thefrontalregion.Noothersymptomsorsignswerepresent; no history of fever,weight loss or sweating was reported. All bloodtests werenegative.Aradiography showed struc-turalalterationofthedistaldiaphysisoftheradius, consist-inginalternatedareasofhyperlucencyandsclerosis, associ-atedwithfocalinterruptionofthecortex(Fig.1aandb).The patientwasadmittedtothePediatricOrthopedicdepartment forfurtherinvestigations.Consideringtheradiography find-ings,CTwas performed.CTexaminationconfirmeda7-cm longstructuralalterationofthedistaldiaphysisoftheradium withamultilayeredperiostealreaction(lamelatedor“onion skin” periostealreaction),withbonerarefactionwithinthe le-sion,withoutinvolvementoftheepiphysis,jointandadjacent bones.ACTofthefrontalregionwasperformedshowinga secondosteolyticlesion,withlenticularmorphology, superfi-ciallyspreadingintosofttissuesanddeeplyintothe subdu-ralbone,withenhancementafteriodinatecontrastmedium administration(Fig.2a-d).A1.5TMRIwasperformedto evalu-atetheradiallesionthatappearedmarkedlyhypointenseon T1-weightedimagesandslighthyperintenseonT2-weighted images.There wasalsoperiosteal reactionwith softtissue edema. There was no soft-tissuemass (Fig. 3a-c).A Tech-netiumbonescanwasperformedforstagingofthedisease. Itshowedfocaluptakesintheradiallesionandinthefrontal bone(Fig.4).AdditionalinvestigationincludingwholebodyCT withcontrastmediumadministration,spinaltap,abone mar-rowaspirationandbiopsywereallnegative.Anopensurgical biopsyoftheradiallesionwasperformed,revealingatypical largecellsdiffuselyinfiltratingthebone. Immunophenotyp-ingshowedpositivityforCD20staining,weakexpressionof BCL2,andnegativityforCD3,CD10,CD30,BCL6,TdT (termi-naldeoxynucleotidyltransferase)andbroad-spectrum cytok-eratins;insituhybridizationforEpstein-Barrvirus wasalso negative.Tumorcellsshowedahighproliferationindex(90%) asdeterminedbyevaluationwithanti-Ki67antibody(Fig.5 a-d).Thepatientunderwentfourcycles ofpolychemotherapy accordingtothenon-Hodgkinlymphoma(NHL)97 protocol (consecutiveblocksofpolychemotherapycontaining dexam-ethasone,cyclophosphamide, methotrexate,vincristine, cy-tarabine,etoposide,ipofosfamide,ara-c,daunomycine,and in-trathecal therapy withmethotrexate, cytarabine,and pred-nisolone).Aftertwocyclesofchemotherapy,thechild under-wentre-stagingthatshowedpartialregressionofbothradial andfrontallesions.Afterthreemorecyclesofchemotherapy, asecondopensurgicalbiopsywasperformed;showing com-pleteremissionofthedisease,and MRIofthefrontalbone

Fig.1– Laterolateralprojection(a)andanteroposterior projection(b)radiographsoftheforearmshowinga structuralalterationofthedistaldiaphysisoftheradius withalternatedareasofhyperlucency(arrows)and sclerosis.Aregularperiostealreactionisevident (arrowheads).

Fig.2– Axial(a)andcoronalreconstruction(b)CToftheforearm.A7-cmlongstructuralalterationofbonerarefactionofthe distaldiaphysisoftheradiumisevident.Multilayeredperiostealreaction(lamellatedor“onionskin” periostealreaction)is present(arrowheads).Thereisnoinvolvementoftheepiphysisjointandadjacentbones.Bonewindow(c)andsofttissue window(d)ofheadCTafteriodinatecontrastmediumadministration,showinganosteolyticarea(arrow)ofthefrontal boneassociatedwithalenticularshapedneoplasticlesion(arrowheads)spreadingintothesofttissue.

Fig.3– MRIoftheforearm,coronalT1(a)andT2 fat-saturated(b)weightedsequences.Theradiallesion

appearedheterogeneouslyhypointenseonT1-weighted

imagesandmainlyhyperintenseonT2-weightedimages.

Thereisnosoft-tissuemass.

Fig.4– Technetiumbonescan.Therearefocaluptakesboth intheradialandinthefrontalbonelesions(arrows).No otherpathologicuptakewasrecognized.

andoftheforearmconfirmedtheabsenceofrecurrence.At 46monthsoffollow-up,thepatientwashealthywithout evi-denceofthedisease.

Discussion

Primary lymphoma of boneis a rare primary bone malig-nancyandisdefinedasalymphomathatisconfinedtothe boneor bonemarrow without evidenceof concurrent sys-temicinvolvement.PLBhasamorefavorableprognosisthan systemiclymphomawithboneinvolvement,with5-year sur-vivalgreaterthan95% [6].Inourcase,norecurrencewas de-tectedafter3yearsoffollow-up.

PLBsitesofboneinvolvementarefemur(27%),pelvis(15%), tibia/fibula(13%),humerus(12%),spine(9%),mandible(2%), radius/ulna(1%),scapula (1%),andskull (1%) [2,3].The ra-diusinvolvementisexceptionallyrare,andDahlin[7]reported only6casesoutofaseriesof905malignantlymphomaof thebone.Themetaphysisisthemostcommonsiteof occur-renceinlongbones.Themostcommonlyaffectedsitesarethe sameforPLB,Ewingsarcoma,osteosarcoma,andtheradiusis araresiteofinvolvementinallofthem[8,9].

TheappearanceofPLBatimagingisusuallyvariableand nonspecific [10, 11] and metastatic lymphoma is indistin-guishablefromprimarybonelymphomawithoutwhole-body stagingtodetectotherdiseaselocalizations.Radiological find-ingsincludewidespreadlyticinfiltrationinthecorticalbone (lytic-destructivepattern70%)orscleroticboneresponse ac-companyinglytic permeation (mixed lytic-sclerotic pattern 28%)asinourcase [10].ThemajorityofPLBpresentaslytic lesion,moreoftenlocalizedinlongbonemetaphysis,withan infiltrativeormoth-eatenpatternofdestructionand aggres-siveperiostealreaction [10,12].Thelesioncanbeassociated

Fig.5– Low(a,c)andhigh(b,d)powerviewofbonebiopsy stainedwithhematoxylin-eosin(a-b)andanti-CD20(c-d). ThemedullaryspacesarefilledbylargeatypicalBcells, stronglypositiveperCD20.

toasofttissuemass [11]asforroundcelltumors(including Ewingsarcoma),amongwhichlymphomaisanexample.On MRI the lesion mostoften haslow signal intensity on T1-weightedimagesanditappearsslightlyhyperintenseon T2-weighted images [11,13].Thebone-marrow involvementis bestdemonstratedonT1-weightedimagesrevealingareasof lowsignalintensitywithinthemarrow,whileonT2-weighted images these areas appear bright. After administration of Gadolinium,T1fat-saturatedsequencescandemonstrate ar-eas ofenhancementwithinthe lesion [10]. Inourcasethe lesionsdidnotshow apermeativepattern whichistypical ofboneinfiltrativeneoplasms,but ratheranirregular corti-caldisruptionwithperiostealreactionandonion-skinpattern. Therewassofttissueedemaandnosofttissuemass.The le-sionswereveryhypointenseinT1-weightedimagesandslight hyperintenseinT2-weightedimages.

Inthefirstdecadeoflife,Ewingsarcomaandosteosarcoma arethemostcommonprimarybonetumors,but malignant lymphomaoftheboneisthethirdmostcommon [7]andit shouldbealwaystakeninconsideration.Metastatic neurob-lastoma,eosinophilicgranulomaandosteomyelitismustbe consideredinthedifferential diagnosistoo.Inourcasethe imagingfindingsand thesymptomsdidnotsuggestthe di-agnosisofEwingsarcomabecauseofthelackofan infiltra-tivepattern,feverand increasederythrocyte sedimentation rate [14].Thehigh-gradeintramedullaryosteosarcomacould mimicPLB. Osteosarcoma commonlypresents with diffuse bonedestructionalternatedtoareasofsclerosisand fociof hemorrhagesandnecrosiscanbeseen.Osteomyelitisinthe earlystagestypicallydemonstrateanosteolyticlesionlocated adjacenttothegrowthplateinthemetaphysisbutwithtime progressivesclerosiscanbeseenaroundthelyticlesion[15].

Insummary,primarylymphomaofboneisararecondition, particularlyinthepediatricpopulation.Theradiographic ap-pearanceofPLBisvariablebutthelytic-destructivepatternis moretypical.AlthoughosteosarcomaandEwingsarcomaare morecommon,asolitarylyticlesioninalongboneshould al-waysraisethesuspicionofPLB.

Supplementary

materials

Supplementary materialassociatedwiththis articlecanbe found,intheonlineversion,atdoi:10.1016/j.radcr.2018.08.025.

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