Case report: Large adrenal ganglioneuroma

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InternationalJournalofSurgeryCaseReports5(2014)253–255

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International

Journal

of

Surgery

Case

Reports

j o u r n al ho m e p a g e :w w w . c a s e r e p o r t s . c o m

Case

report:

Large

adrenal

ganglioneuroma

Cos¸

kun

Kacagan

_,

_Ekrem

_Basaran

_,

_Havva

_Erdem

_,

_Ali

_Tekin

_,

_Ali

_Kayikci

_,

_Kamil

_Cam

a_{DepartmentofUrology,DuzceUniversitySchoolofMedicine,Duzce,Turkey} b_{DepartmentofPathology,DuzceUniversitySchoolofMedicine,Duzce,Turkey}

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Articlehistory:

Received11September2013

Receivedinrevisedform17February2014 Accepted4March2014

Availableonline13March2014

Keywords: Casereport Adrenalmass Incidentaloma Ganglioneuroma

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INTRODUCTION:Ganglioneuromasarelocalizedtumorsderivedfromneuralcresttissues. Characteristi-cally,theyoriginateintheposteriormediastinum.Pureadrenalgangliomasareextremelyrare. PRESENTATION OF CASE: A left adrenal mass with the size of 68mm×50mm×86mm on mag-neticresonanceimagingwasdocumentedina53-year-oldfemalepatient.Endocrinetestsrevealed anon-functioningadrenalmass.The actualsizeofthe masswas macroscopicallymeasuredtobe 16cm×8.5cm×6cmafterthesurgery.Histopathologicalexaminationindicatedganglioneuroma. DISCUSSION:Mostadrenalganglioneuromascanincorrectlybediagnosedasotheradrenaltumors,since theyarerareneurogenicbenigntumorswithnospecificimagingproperties.Theyhaveaslowgrowth patternandusuallyasymptomatic.Ourcaserepresentsahugeadrenalganglioneuromainafemalepatient withnondiagnosticflankpain.Radiologicalimagingshowedalargeadrenalmasswithnodifferentiation fromotheradrenaltumors.Endocrineevaluationshouldbeperformedforsuchadrenalmasses.Sinceour casehadarelativelylargesize,opensurgerywaspreferred.Pathologyrevealedthedefinitivediagnosis. CONCLUSION:Thiscasesuggeststhatganglioneuromascanwronglybediagnosedasotheradrenaltumors. Itissignificantthataproperdifferentialdiagnosisshouldbeperformedbyusinghormonalandimaging techniques.Nevertheless,pathologicalexaminationisusuallyrequiredfordefinitivediagnosis.

©2014TheAuthors.PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.Thisisanopen accessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/3.0/).

1. Introduction

Adrenalincidentalomasarerare,small,andinsidentalmasses.1

In thecase ofanadrenal incidentaloma, benign/malignant,and functioning/non-functioning distinctions should be carried out. The most frequently reported one is the adrenal cortical ade-nomawithaprevalenceof36–94%.2_{Adrenalmassesareclassified}

accordingtotheirorigin.Pheochromocytoma,ganglioneuromaand neuroblastomaarederived fromtheadrenalmedulla.Adenoma, myelolipoma,andadrenocorticalcarcinomaarederivedfromthe adrenalcortex.Furthermore,adrenalincidentalomasare catego-rizedaccordingtotheirhormonalactivities.Thevastmajority(85%) ofadrenalincidentalomasarenonfunctioning.Whenexaminedfor hormonesecretion,20%ofthecasesexhibitsubclinical dysfunc-tion.Ofthese,9%havesubclinicalCushing’ssyndrome,and1%has highlevelsofaldosterone.Highcortisolandaldosteronelevelsare usuallyencounteredinthecasesofbenignadenomas.Moreover,in thepresenceofsexsteroids,amaligntumorshouldbeconsidered.

∗ Correspondingauthorat:DepartmentofUrology,DuzceUniversitySchoolof Medicine,Konuralp,81620Duzce,Turkey.Tel.:+903805421390;

fax:+903805421387.

E-mailaddress:ckacagan@gmail.com(C.Kacagan).

Ganglioneuromas are tumors derived from neural crest tis-sues,whicharemostlylocalizedintheposteriormediastinumand retroperitoneal region.Pureadrenal ganglioneuromas are espe-ciallyrare.3_{Ganglioneuromascompriseabout0–6%ofallofadrenal}

incidentalomas.4_{Adrenalganglioneuromascanstayasymptomatic}

untiltheyreachalargesize,andareusuallyincidentallydiagnosed withimaging techniques.Sometimes radiologicalinvestigations canbeperformedasaresultofnonspecificsymptomscausedby pressure.5_{Adrenalganglioneuromascaninitiallybemisdiagnosed}

asadrenalcarcinomaandpheochromocytoma.Therefore,aproper differentialdiagnosispriortosurgeryisvital.Inthiscase presenta-tion,weincidentallydiscoveredalargeadrenalganglioneuromaon magneticresonanceimaging(MRI)ina53-yearoldfemalepatient.

2. Case

A53-yearoldfemalepatientwasadmittedtoourclinicwith complaintsofnonspecific,non-colic,intermittentbilateralflank pain,predominantlyontheleftside.Shehad painforoneyear. Thepatient’shistorydidnotcontainanysystemicdiseasesor pre-viousoperations.Physicalexaminationrevealednoabnormality. Anon-contrastabdominalCT(computedtomography)showeda 75mm×50mmlobulatedcontourmasslesionintheleftadrenal gland.TheMRIrevealeda68mm×50mm×86mm,mostly well-circumscribed,heterogeneous solidlesion withaslight contour

http://dx.doi.org/10.1016/j.ijscr.2014.03.004

2210-2612/© 2014TheAuthors. Publishedby ElsevierLtd.on behalfof SurgicalAssociates Ltd.Thisis an openaccessarticle underthe CCBY-NC-ND license

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254 C.Kacaganetal./InternationalJournalofSurgeryCaseReports5(2014)253–255

Fig.1.MRIimageoftheadrenalmassof68mm×50mm×86mm.

irregularity ontheleft adrenal (Fig.1).The mass showed pro-gressivecontrastincreaseindynamicexaminations.Consequently, lesionwasevaluatedtobe“non-adenoma”,andwasreportedasa metastaticlesionoradrenocorticalcarcinoma.

Serumcortisol,DHEA-SO4,urinecortisollevelswerefoundto be1.56_␮g/dl,88.48_␮g/dl,16.89_␮g/dlrespectively,andallin nor-malranges.Dexamethasone1mgsuppressiontestwasperformed. Thelesionwasnon-functionalaccordingtothisendocrine eval-uation.Open leftsurrenalectomy(totalsurgicalresectionofthe adrenalgland)wasperformed.Patienthadnocomplaintsafterthe operationandshewaswellatthefollow-upvisitafter1year.

Duringmacroscopicexamination,theweightand sizeofthe massweremeasuredtobe227g,and16cm×8.5cm×6cm. Mark-ersusedwereS100andsmoothmuscleactin(SMA).Pathological examinationrevealedmatureganglioneuromahavingfasciclesof Schwann-likecells(Fig.2a)withpositivestainingofS100(Fig.2b) and SMA. These Schwannian cells constituted the majority of

Fig.2.(a)Pathologicalinvestigationshowsthatthestromaisfurnishedwith fas-ciclesofSchwann-likecells.Alsoganglioncellsformmatureclusters.Hematoxylin andeosin,40×.(b)PositiveimmunohistochemicalstainingwithS100,200×.

stroma.Thespecimencomposedofganglioncellswithnoresidual neuroblasts.Thelargestdiameterofthetumorwas16cmwith neg-ativesurgicalmargins,andgrosslynodistincthemorrhagicnodules wereobserved.

3. Discussion

Arecentstudystatesthatpureadrenalgangliogliomasarevery rareinJapan,andonly4caseshavebeenreported.3_Adrenal

gan-gliogliomashaveaslowgrowthpatternandusuallyasymptomatic. Ourcaserepresentsahugeadrenalganglioneuromainafemale patientwithnonsdiagnosticflankpain.Interestinglyinitial ultra-sonographymissedtheadrenalmass.Therefore,suchpatientswith persisted flank pain a careful evaluation should beconsidered. Anappropriateultrasonographicexaminationincludingadrenals shouldbeperformedandevenCTcanbeusedindifferential diag-nosis.Thelargestganglioneuromareportedtodatewas19cm.6

Our case had a relatively large tumor as 16cm at its largest diameter.

Ganglioblastomaandneuroblastomaarealsopartsofthisclass. Ganglioneuroma can be distinguished from these two tumoral masseswiththepresenceofmaturesympatheticganglioncells. Ganglioneuromas are hormonally nonfunctioning tumors, but sometimesmay beassociated withpheochromocytoma. In this case,theycansecretecatecholaminesandtheirmetabolites.Rarely, somestudiesintheliteraturealsosuggestthat,ganglioneuromas cansecretecortisolandandrogen.7 _{Itwasassertedthatmasses}

whichareunder3cmhaveverylowprobabilityofbeingfunctional, and the onesabove 3cm are most likely functional. Therefore, endocrineevaluationshouldbeperformedforsuchlargeadrenal masses. Additionally, whenan adrenal mass isdiscovered, 75% ofthetime, thecauseismetastasizes fromotherorgans.These areprimarilykidney,colon,breast,esophagus,pancreas,liverand stomach.Adrenalmassesofmetastasisareusuallybilateral.

MRIandCTviewsofganglioneuromascanmatchthoseofother adrenaltumors. Inotherwords,whenonly focusedonimaging techniques,itisclearthatganglioneuromacannotbedistinguished from adrenocortical carcinoma and pheochromocytoma. In all casesofadrenalincidentalomas,urinemustbemonitoredfor cat-echolamineand fractionatedmetanephrinefor 24hprior tothe operation. Theincreasesin levelsofmetanephrineand/or cate-cholamineindicatethatthistumoralmassispheochromocytoma witha91%and98%ofsensitivityandspecificity,respectively.In termsofdifferentialdiagnosistoruleoutmalignancy,fine-needle aspirationbiopsyhasahighrateoffalsenegativity.Inaddition, somecomplicationsofbiopsyarealsopresent.Alongwiththese, primarytreatmentmodalityinadrenocorticalcarcinomaisopen orlaparoscopicsurgicalexcision.Sinceourcasehadrelativelylarge size,opensurgerywaspreferred.

Tumorsizescanbemisidentifiedataboutarateof 20%and 16–47%forMRIandCTrespectively;justasinourcase.8_Tumorsize

isveryimportanttodeterminetheprognosisforadrenalmasses. Statistically,tumoralmassesbelow4cmare60%benignadenomas, andcarrya2%riskofadrenalcorticalcarcinoma.Inthecasethatthe tumorislargerthan6cm,25%chanceofadrenocorticalcarcinoma isexistent.Therefore,itisnotappropriatetomakeamalign/benign decision solelybased onsizesreported viaimaging techniques. Mostlypathologicalinvestigationprovidedthedefinitivediagnosis ofganglioneuroma.Arecentpublicationof29ganglioneuroma con-firmedthathistopathologicaldiagnosisisessentialandsurgeryis themaincurativemethod.9_{However,anotherrecentreportof27}

patientsshowsthatrarelylocalextensionormalign transforma-tionispossibleforganglioneuroma.10_{Therefore,surgeryshould}

beofferedforthesepatients.Moreover,postoperativefollow-up shouldnotbeignored.

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C.Kacaganetal./InternationalJournalofSurgeryCaseReports5(2014)253–255 255 Duetothefactthatganglioneuromascanbeerroneously

diag-nosedasotheradrenaltumors, itis imperativethat differential diagnosisshouldbeperformedbyusingappropriatehormonaland imagingtechniques. Conflictofinterest None. Funding None. Ethicalapproval

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.Acopy ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief ofthisjournalonrequest.

Authorcontributions

Cos¸kunKacagan:datacollections,writing;EkremBasaran:data collections;HavvaErdem:datacollections;AliTekin:studydesign,

datacollections;AliKayikci:dataanalysis,writing;Kamil Cam: studydesign,writing.

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