Retroperitoneal paraganglioma presenting with pancytopenia: A rare case with rare manifestation

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InternationalJournalofSurgeryCaseReports14(2015)77–79

ContentslistsavailableatScienceDirect

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

Retroperitoneal

paraganglioma

presenting

with

pancytopenia:

A

rare

case

with

rare

manifestation

Mutlu

Ünver

_,

_S¸

_afak

_Öztürk

_,

_Varlık

_Erol

_,

_Erdem

_Barıs¸

_Cartı

_,

_Osman

_Bozbıyık

_,

Eyüp

Kebapc¸

ı

_,

_Mustafa

_Ölmez

_,

_Gökhan

_Akbulut

a_{˙IzmirUniversity,DepartmentofGeneralSurgery, ˙Izmir,Turkey} b_{Bas¸kentUniversity,DepartmentofGeneralSurgery, ˙Izmir,Turkey} c_{AydınStateHospital,DepartmentofGeneralSurgery,Aydın,Turkey} d_{AfyonKocatepeUniversity,DepartmentofGeneralSurgery,Afyon,Turkey}

e_{TepecikEducationandResearchHospital,DepartmentofGeneralSurgery, ˙Izmir,Turkey}

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Articlehistory: Received2July2015

Receivedinrevisedform18July2015 Accepted20July2015

Availableonline28July2015

Keywords: Paraganglioma Pancytopenia Retroperitoneal Reactivethrombocytosis

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INTRODUCTION:Paragangliomas aretumorsthatarisefromextraadrenalchromaffincellsand most ofthemareasymptomaticpresentingwithpainlessmass.Retroperitonealparagangliomasaremostly benignwithgoodprognosis;however,theycanpresentwithabdominalpain,palpablemass,or hyper-tensiveepisodes.Surgicalresectionisstillthemaintreatmentandnecessaryforhistologicalassessment. CASEREPORT:A41yearoldfemalepatientpresentedwith6monthsoflossofappetite,weightloss, weaknessandbreathlessnessonexertion..Thepatient’sinitialbloodexaminationshowedmarked ane-mia,reducedleukocytecountwithneutropeniaandlymphopeniaandamarkedreductionintheplatelet count.Thepatientwasadmittedforevaluationofherpancytopenia.Magneticresonanceimagingrevealed a8×7×8cmsizedmassclosedtothepancreatictailinvadingsplenichilum.Alargemasswasidentified retroperitoneally,closedtothetailofpancreaswithasplenichiluminvasion.Totalmassresectionand splenectomywasperformed.

DISCUSSION:Completesurgicalexcisionisthetreatmentofchoiceforextra-adrenalparagangliomasas wellasforrecurrentormetastaticneoplasms.Reactivethrombocytosisisacommoncauseof thrombocy-tosis.Splenectomywasfoundtobeoneofthemaincausesofextremereactivethrombocytosis.Reactive thrombocytosisisapredictablefindingaftersplenectomyandmanagementofthethrombocytosisand preventionofcomplicationsshouldbeinitiated.

©2015TheAuthors.PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.Thisisanopen accessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

1. Introduction

Paragangliomasaretumorsthatarisefromextraadrenal chro-maffincellsandcandevelopeatvarioussitesofthebodyincluding thehead,neck,thoraxand theabdomen.Mostof the paragan-gliomasareasymptomaticpresentingwithpainlessmass.About 75%ofparagangliomasare sporadicand 85%ofparagangliomas developintheabdominalcavity[1].

Whenpresentingwithintheabdominalcavity,theymayariseas aprimaryretroperitonealneoplasmandcanmimicvascular mal-formationsorotherconditionsrelatedtospecificretroperitoneal organssuchasthepancreas,kidneys,oradrenals.Retroperitoneal paragangliomasarisefromspecializedneuralcrestderived cells distributed alongthe aortainassociationwiththesympathetic

∗ Correspondingauthorat: ˙IzmirUnivercity,DepartmentofGeneralSurgery,Yeni GirneBulv.1825skno.12,Kars¸ıyaka, ˙Izmir,Turkey.Fax:+902323423371.

E-mailaddress:mutluunver@gmail.com(M.Ünver).

chain.Retroperitonealparagangliomasmainlyaffectadultswho are in the fourth or fifth decade of life, and they have nosex predilection[2].Retroperitonealparagangliomasaremostlybenign withgood prognosis; however, theycan present with abdomi-nal pain, palpable mass, or hypertensive episodes [3]. Patients withsecretorytumorsexperienceparoxysmalepisodic hyperten-sion, as wellas the typical triad of symptoms associated with pheochromocytomas, for example, palpitations, headache and sweating.Nonfunctionalparagangliomasmostcommonlymanifest asabdominalpainoramass[4].Inthepresentcase,wereporta rarecaseofaretroperitonealparagangliomapresentingwith pan-cytopenia.

2. Casereport

A41yearoldfemalepatientpresentedwith6monthsofloss of appetite,weightloss,weakness,and breathlessnesson exer-tion.Therewasnohistoryofsmokingandalcohol abuse.There wasnoremarkablefamilyhistory.Onadmission,vitalsigns(blood

http://dx.doi.org/10.1016/j.ijscr.2015.07.021

2210-2612/©2015TheAuthors.PublishedbyElsevierLtd.onbehalfofSurgicalAssociatesLtd.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://

creativecommons.org/licenses/by-nc-nd/4.0/).

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78 M.Ünveretal./InternationalJournalofSurgeryCaseReports14(2015)77–79

Fig.1.T2weightedaxialMRIimage(redarrowshowsthespleenandyellowarrow showsthemasswhichiscentrallyhyperintenseandperiferallyhypointense).(For interpretationofthereferencestocolorinthisfigurelegend,thereaderisreferred tothewebversionofthisarticle.)

pressure,heartrate,respirationrate,andbodytemperature)were withinnormallimits.Therewasnoperipherallymphadenopathy, hepatomegalyorsplenomegalyinphysicalexamination.Theinitial bloodexaminationshowedmarkedanemia(Hb=7.6g/dl),reduced leukocytecount(2100/cumm)withneutropeniaandlymphopenia andamarkedreductionintheplateletcount(70,000platelets/cu mm).Arepeatcompletebloodcount(CBC)showedsimilarresults. Basic metabolic panel,chest X-ray film,and electrocardiogram resultswereallnormal.Tumormarkerassaysshowedthat alpha-fetoproteinwas7.2n/ml(normal0–8.1),carcinoembryonicantigen (CEA)was3ng/ml(normal0–5),carbohydrateantigen19–9(CA 19–9)was11U/ml(normal0–37).

The patient was admitted for evaluation of her pancytope-nia by haematology department. All the results, testing for humanimmunodeficiencyvirus(HIV), Epstein–Barrvirus(EBV), cytomegalovirus,hepatitis,parvovirusB19,andantinuclear anti-bodieswerenegative.B12andfolatelevelswereinnormallimits. Upper GI endoscopy and colonoscopy were also normal. Then bonemarrowaspirationwasperformedtoevaluatethecauseof pancytopenia.Theaspiratewasnormocellularandshowed nor-malmaturationofallthethreeseries.Thebonemarrowreaction wasnormoblastic.AbdominalUSGwasperformedtorevealedthe dimensionsofthespleen.A 8×7sizecm massfrompancreatic tailtosplenichilumwasobservedintheabdominalUSG. Mag-neticresonanceimagingrevealeda8×7×8cmsizedmassclosed tothepancreatictailinvadingsplenic hilum(Fig.1).Thepatient underwentalaparotomywithamediansuperiorincision.Alarge masswasidentifiedretroperitoneally,closedtothetailofpancreas withasplenicveinocclusion.Totalmassresectionand splenec-tomywasperformed(Fig.2).Meanoperativetimewas120minand meanbloodlosswas200cc.Asignificantimprovementinthetotal leucocyteandplateletcountwasseenonthethirddayofsurgery. Therewasanincreaseinthehaemoglobinconcentrationbutwas thelastparametertoshowimprovement.Herplateletcountwas abnormallyelevatedto677,000platelets/cummpostoperativeday 6.Thepatientremainedasymptomaticatdischarge,andthe recom-mendationsfor reactivethrombocytosisaftersplenectomywere discussedwithher.Werecommendedmobilizationandincreased fluidintake.Shewasstartedon100mgofaspirindaily.Thepatient

Fig.2. Grosslycharacteristicbrownappearenceofthetumor.Tumormassis well-surcumscribedwithafibrouscapsule.

wasdischargedafter7postoperativeday.Onemonthfollowing surgery,bloodcountswereinnormallimits.Pathological exami-nationofthespecimenwasreportedasparaganglioma(Fig.3).

At 6-months follow-up, the patient’s complete blood count remainsnormalandabdominalCTdidnotrevealanyrecurrence ofthemass.

3. Discussion

Paragangliomas are extra-adrenal chromaffin tumours that developattheexpenseofneuroectodermalcellsoftheautonomous nervoussystem[5].Paragangliomascandevelopeatvarioussitesof thebodyincludingthehead,neck,thoraxandtheabdomen.Despite mostoftheparagangliomasareasymptomatic,retroperitonealand nonfunctioningformsareveryrare[6].Thefirstimagingmodality inthediagnosisofextra-adrenalparagangliomasismagnetic reso-nanceimaging(MRI)secondarytosuperiortissuecharacterization and absence of radiation hazards [7]. Computerized tomogra-phy(CT)scanalsohassensitivityof around90%for identifying extra-adrenalparagangliomasand theyappearas highly vascu-larstructurewithareasofintralesionalhemorrhageandnecrosis [8]. Metaiodobenzylguanidine scintigraphy (MIBG scintigraphy) hasoftenbeenusedasanimagingmodalityin thediagnosisof neuroendocrinetumors,butitlackssensitivityforextra-adrenal paragangliomas[1].

Completesurgicalexcisionisthetreatmentofchoicefor extra-adrenal paragangliomas as well as for recurrent or metastatic neoplasms[2].Patientwithmetastaticdiseasewillrequire adju-vantradiotherapywhilechemotherapyisrestrictedtopatientsnot accessibleforsurgeryandresistanttoradionuclidetherapy[9].

Reactivethrombocytosisisacommoncauseof thrombocyto-sis[10].Splenectomywasfoundtobeoneofthemaincausesof extremereactivethrombocytosisasinthepresentcase[11]. Reac-tivethrombocytosisisapredictablefindingaftersplenectomy,with theplateletcountpeakingat1to3weeksandreturningtonormal levelsinweeks,months,and,rarely,years[12].Essential throm-bocytosisisnot awell-recognizedcauseforarterialandvenous thrombosis.Managementofthethrombocytosisandpreventionof complicationsshouldbeinitiated[13].Thefirstlineoftherapyis theadministrationofplatelet-antiaggregatingmedicationsuchas aspirin[14].

Histopathologicalfindingsare notmuch useful to differenti-atebetweenbenignandmalignantparagangliomasonlyextensive localinvasion and distant metastasistoliver,bone, and lymph nodeshavebeenusedasindicatorsformalignancy[1,8]. Follow-up imaging is necessary in cases of patients with elevated

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M.Ünveretal./InternationalJournalofSurgeryCaseReports14(2015)77–79 79

Fig.3.(A):ChromograninA:TumorcellsarediffuselypositiveforneuroendocrinemarkerChromograninAimmunohistochemically.(B):H&E:Welldefinednestsofcuboidal cellsseparatedbyhighlyvascularizedfibroussepta.Individualcellshaveamoderatelyabundantgranularbasophiliccytoplasm.

metanephrine and catecholaminelevelsor withnon functional original tumors [3]. Because of malignant potential and higher recurrencerateinparagangliomas,lifelongfollowupisalways rec-ommended[15].

4. Conclusion

Paragangliomas are very rare entities and has never been reported beforemanifesting withpancytopenia. Surgical resec-tion is still the main treatment and necessary for histological assessment.Reactivethrombocytosisisapredictablefindingafter splenectomyasinthepresentcase.Managementofthe thrombocy-tosisandpreventionofcomplicationsshouldbekeptinmindafter splenectomy.

Funding None. Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandanyaccompanyingimages. Ethicalapproval

N/A.

Conflictofinterest None.

Authorcontribution

MutluÜnverM.D.;Studyconceptordesign,datacollection,data analysisorinterpretation,writingthepaper.

S¸afakÖztürkM.D.;Studyconceptordesign,datacollection,data analysisorinterpretation,writingthepaper.

VarlıkErol M.D.;Dataanalysis orinterpretation,writingthe paper.

ErdemBarıs¸CartıM.D.;Dataanalysisorinterpretation,writing thepaper.

OsmanBozbıyıkM.D.;Dataanalysisorinterpretation,writing thepaper.

EyüpKebapc¸ıM.D.;Studyconceptordesign,datacollection. MustafaÖlmezM.D.;Studyconceptordesign,datacollection. GökhanAkbulutM.D.;Studyconceptordesign,datacollection, dataanalysisorinterpretation,writingthepaper.

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