Introduction SclerosingangiomatoidnodulartransformationSANTisavery rare benignlesion in the spleen, initiallydescribed in2004 by Marteletal.[1].Sofarfewerthan100casesweredocumented, butd
Trang 1Department of Gastroenterological Surgery, Hiroshima City Hiroshima Citizens Hospital, 7-33 Motomachi, Naka-ku, Hiroshima, Japan
a r t i c l e i n f o
Article history:
Received 14 November 2016
Received in revised form
28 November 2016
Accepted 28 November 2016
Available online 30 November 2016
Keywords:
Sclerosing angiomatoid nodular
transformation (SANT)
Adrenalectomy
IgG4-associated disease
Glucocorticoid
Hand assisted laparoscopic surgery
a b s t r a c t
INTRODUCTION:Sclerosingangiomatoidnodulartransformation(SANT)isararebenignlesionwithan unknownnaturalhistoryandpathogenesis.Sofarfewerthan100casesweredocumented,butdetailed incidenceandprevalenceareunknown
PRESENTATIONOFCASE:WereportacaseofSANTofthespleenina37-year-oldmanthatshowedrapid growthafteradrenalectomyforprimaryaldosteronism.Computedtomographyshowedanoduleinthe spleenthatincreasedinsizefrom2.0cmto7.0cmduring3yearsofobservation
DISCUSSION:Thiscaseisreportedbecausedataregardinggrowthratesandnaturalhistoryoftheselesions arelimitedandfewcaseshavebeenreportedtoshowtherapidgrowthprogressionseeninthiscase CONCLUSION:Decreasesinglucocorticoidconcentrationsfollowingadrenalectomymayhavecontributed
totherapidgrowthofSANTofthespleen,becauseSANTisconsideredtoberelatedtoimmunoglobulin G4-associateddisease
©2016TheAuthor(s).PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.Thisisanopen accessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/)
1 Introduction
Sclerosingangiomatoidnodulartransformation(SANT)isavery
rare benignlesion in the spleen, initiallydescribed in2004 by
Marteletal.[1].Sofarfewerthan100casesweredocumented,
butdetailedincidenceandprevalenceareunknown.SANTis
com-posedofangiomatoidnodulesinafibroscleroticbackground[2]
Histologically,theangiomatoidnodulescompriseseveraltypesof
bloodvesselsdistinguishedbytheirimmunohistochemicalprofiles,
includinga cordcapillary-like type (CD31+CD34+CD8-), small
veins(CD31+CD34-CD8-),andasinusoid-liketype(CD31+
CD34-CD8+)[1,3]
SANTappearstofollowabenignclinicalcourse,with
splenec-tomybeingcurative,butdataregardingthenaturalhistory and
growthratesofthisdiseasearescarce.ThepathogenesisofSANT
alsoremains unclear.SANThasbeenhypothesizedtorepresent
apeculiarhamartomatoustransformation ofsplenicredpulpin
responsetoexaggeratednon-neoplasticstromalproliferation[3]
Ontheotherhand,SANTisreportedlyassociatedwithEpstein-Barr
virus(EBV)infection[2],andimmunoglobulin(Ig)G4-related
scle-rosingdisease[5,6].WereporthereinacaseofSANTofthespleen
presentingrapidgrowthafteradrenalectomyforprimary
aldos-teronism.Wechosesplenectomy byhandassisted laparoscopic
surgery(HALS),didnotperformminimalinvasivesurgerysuchas
∗ Corresponding author.
E-mail address: ddds4863@gmail.com (D Satoh).
tumorectomy,becausewecouldnotcompletelydenyamalignant lesion
WediscussthepossiblepathogenesisandtreatmentofSANT
2 Case report
A33-year-oldman wasadmittedfor SANT.Hismedical his-toryhadlaparoscopicadrenalectomyforprimaryaldosteronism Physical examination of the abdomen showed no abnormali-ties Abdominal computed tomography (CT) performed 1year after surgery incidentally revealed a hypodense splenic lesion (20mm)(Fig.1).Physical examinationoftheabdomenshowed
noabnormalities.Laboratoryexaminationsincludingserum car-bohydrateantigen(CA)19-9andcarcinoembryonicantigen(CEA) concentrations were within normal limits Plain CT showed a low-density,circularlesionwithawell-circumscribedborder.No cysticchanges,necrosisorcalcificationwasevidentinthelesion Contrast-enhancedCTshoweddelayedenhancementcentripetally, with a wheel-like appearance Abdominal magnetic resonance imaging(MRI) demonstratedanoduleinthespleenwithsignal hypointensityonT1-weightedimaging,andheterogeneoussignal hyperintensityonT2-weightedimaging(Fig.2)
Themainradiologicaldifferentialdiagnoseswerehamartomas, sclerosedhemangioma,andinflammatorypseudotumor.Asnosign
ofmalignancywaspresent,wedecidedtoobservetheprogress
ofthelesionwithoutintervening.Twoyearslater,CTshowedthe masswasappearedtobeprogressive(50mm).Thenextyear,the masshadincreasedinsizeto70mm(Fig.1).Becausemalignant
http://dx.doi.org/10.1016/j.ijscr.2016.11.054
2210-2612/© 2016 The Author(s) Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd This is an open access article under the CC BY-NC-ND license ( http://
Trang 2Fig 1. Abdominal computed tomography (CT) a) A 2.0-cm mass is evident in the spleen 1 year after adrenalectomy b) Three years after adrenalectomy, the mass had increased in size to 5 cm c) The next year, the mass has increased in size to 70 mm.
Fig 2. Magnetic resonance imaging (MRI) a) A signal-hypointense nodule is seen in the spleen on T1-weighted imaging b) Heterogeneous signal hyperintensity is seen on T2-weighted imaging.
neoplasmcouldnotbecompletelyexcludedgivensuchprogressive
growth,splenectomywasperformed
Wechosehandassistedlaparoscopicsurgery(HALS)bylateral
decubitusposition,becausethetumorlocatedatthespleenandit
wasnotinvasive.(Fig.3)
Grossexaminationrevealedawell-demarcated,solitarylesion
measuring70×65mm,containingacentralbulkystellatemassof
whitecompacttissue(Fig.3).Histopathologicalfindingsshowed
three types of vascular structure in the tumor: capillary-like
vessels; dilated sinusoid-like vessels; and ectatic veins
Nei-thernuclear atypianornecrosis waspresent Immunochemical
analysisrevealedacomplexvascularpattern,inwhichthe
capil-lariesshowedCD34+/CD8-/CD31+,thedilatedsinusoid-likevessels
showedCD34-/CD8+/CD31+,andsmallveinsshowed
CD34-/CD8-/CD31+.Inthescleroticstroma,someplasmacellsrevealedaIgG4+
status(Fig.4).EBVwasnotdetectedaccordingtoEBV-encodedRNA
(EBER).ThepathologicdiagnosiswasSANT
3 Discussion
SANTofthespleenisrareandmostlyrecognizedasanincidental findingonroutinemedicalimaging,asinthiscase.Thepatientsare usuallyasymptomaticbutsometimesanabdominaldiscomfortor paincanbeobserved.Zhixinetal.reviewed127patientswithSANT
Inthisreport,aslightfemalepreponderanceisevident,and pre-dominantlyaffectsmiddle-agedadultsaccordingtoareview[7] Althoughtheexactnatureofthisdiseaseremainsunclear,SANTis consideredbenignanddoesnotpresentwithrecurrenceor malig-nantbehavior[8].Inourcase,thespleniclesiondisplayedarapid growthinsizefrom20mmto70mmduring3yearsafter adrenalec-tomy.Surprisingly,CTshowednolesioninthespleenjustbefore adrenalectomy,andCT1yearafteradrenalectomyrevealeda
20-mmlesioninthespleen.AlthoughgrowthofSANTonfollow-up imagingisreportedlycommon[9],fewreportshaveshownsuch rapidgrowthasseeninthepresentcase.Ofcourse,thesefindings
Trang 3Fig 3.Gross examination reveals a well-demarcated, solitary lesion, measuring 70 × 65 mm, which contains a central bulky stellate mass of white compact tissue.
Fig 4. Immunohistochemical examination reveals positive staining for IgG4
(orig-inal magnification, ×200).
mayhavebeencoincidental,butthepossibilitythatadrenalectomy
facilitatedtheimpressivegrowthdynamicsofSANTmustbe
con-sidered
ThepathogenesisofSANThasnotbeenfullyelucidated.Martel
etal.speculatedthatSANTmayrepresentapeculiar
hamartoma-toustransformationofsplenicredpulpinresponsetoexaggerated
non-neoplasticstromalproliferation[1].Suchtransformationmay
resultinsmallvesseloutflowfailure,leadingtonodularor
hyper-plasticchangesoftheproximalvasculartract.Zhangetal.proposed
thatSANTrepresentsavascularmalformationratherthan
heman-gioma[10],becauseimmunochemicalstainingofspecimensfrom
SANT showednegative results for glucose transporter 1 SANT
haspreviouslybeenreportedtobeassociatedwithEBVinfection
[11,12].Ontheotherhand,SANTisconsideredtoberelatedtoIgG4
associateddisease[13–15].Thepresenceofasignificantlyhigher
numberofIgG4+plasmacellsandanincreasedIgG4/IgGratiohas
beenreportedinsomestudies[13–15].Inourcase,EBVwasnot
detectedbyEBER,andalthoughwedidnotmeasureserumIgG4
levels,immunochemicalstainingforIgG4yieldedpositiveresults,
supportingthepossibilitythatSANTwasrelatedtoIgG4-associated
disease.IgG4-associateddiseaseisthetermusedtorefertoa
condi-tioncharacterizedbylymphoplasmacyticinfiltration,fibrosisand
increasednumbersofIgG4+cellspresentintissue[16].Thisdisease
hasanallergicbackgroundandisimmune-mediated.Up-regulated
responsesofThelper2andTregulatorycellsandtheassociated
cytokinesplaymajorroles indiseaseprogression [17],and
fre-quentlyaffectthepancreas,salivaryglandsandlymphnodes,but
caninvolvealmostanytissue.Nointernationalconsensushasbeen reachedregardingdiagnosticcriteriaforthedisease,butthetypical histopathologyis lymphoplasmacyticinfiltration withabundant IgG4-positive plasmacells Elevated serum IgG4concentrations supportthediagnosis.Thetreatmentofchoiceiscorticosteroids
Inthiscase,serumcortisollevelsdecreasedfrom21.4ng/dlbefore adrenalectomyto4.0ng/dlafteradrenalectomy.We would like
toemphasize thattherapid growthof thelesionin thespleen wasconsistentwiththetimeperiodinwhichserumcortisol lev-elsdecreased, which mightindicate thatrapid growthofSANT resultedfromdecreasedserumconcentrationsofcortisol Consid-eringthat SANTofthespleen canberelated toIgG4-associated disease,corticosteroidsmightbeusefulfortreatingSANT.While objectiveevidenceislackingandthispossibilityremainsnobetter thanahypothesis,webelieveourfindingsarehelpfulforpossible treatmentofpatientswithSANT
Inconclusion,wehavereportedthecaseofauniquepatientwith rapidgrowthofSANTafteradrenalectomy.SANTisconsideredto showsomerelationshiptoIgG4-relateddisease,socorticosteroids maybeeffective,supportedbyourfindings.However,further stud-iesareneededtoelucidatethepathogenesisofSANT
Conflict of interest statements
DaisukeSatohandco-authorshavenoconflictsofinterestto declare
Funding
Wehavenosourcesoffunding
Ethical approval
Iconfirmethicalapprovalhasgiven
Consent
IconfirmthatIhaveobtainedsignaturetopublishacasereport fromthepatient
OurworkhasbeenreportedinlinewiththeSCAREcriteria
Author contribution
Nospecificcontributions
Registration of research studies
Thisisnotresearch
Trang 4[5] S Kashiwagi, T Kumasaka, N Bunsei, Y Fukumura, S Yamasaki, K Abe, et al.,
[16] O Ardila-Suarez, A Abril, J.A Gómez-Puerta, IgG4-related disease: a concise review of the current literature, Reumatol Clin (2016), http://dx.doi.org/10.
OpenAccess
ThisarticleispublishedOpenAccessatsciencedirect.com.ItisdistributedundertheIJSCRSupplementaltermsandconditions,which permitsunrestrictednoncommercialuse,distribution,andreproductioninanymedium,providedtheoriginalauthorsandsourceare credited