Idiopathic granulomatous mastitis: A report of twenty cases

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ORIGINAL ARTICLE / Senology

Idiopathic granulomatous mastitis: A report of twenty cases

Houssine Boufettal

^a^,∗

, Fatiha Essodegui

^b

,

Mohammed Noun

^a

, Saïd Hermas

^a

, Naïma Samouh

^a

aDepartmentofObstetricsandGynaecologyC,IbnRochdUniversityHospital,Schoolof MedicineandPharmacy,AïnChokUniversity,Casablanca,Morocco

bCentralRadiologyDepartment,IbnRochdUniversityHospital,SchoolofMedicineand Pharmacy,AïnChokUniversity,Casablanca,Morocco

KEYWORDS Breast;

Inﬂammation;

Granuloma;

Idiopathic

Abstract

Introduction:Idiopathicgranulomatousmastitisisabenignlesionofthemammaryglandchar- acterisedbythepresenceofnon-infectiousinﬂammatorybreastlesionslimitedtothelobules.

Objective:Wereporttwentycasesofidiopathicgranulomatousmastitis(IGM)withadiscussion ofepidemiology,clinicalanddiagnosticfeatures,treatmentandprogressofthispathological entity.

Materialsandmethods:Aretrospectivestudyoftwentycasescompiledfromaten-yearperiod, from952pathologicalanatomyexaminationscarriedouttoinvestigatevariousbreastpatholo- gies.

Results:Thepatientshadameanageof45.5years.Clinicalexaminationsrevealedatumefac- tionmeasuringbetween2.5and18cmindiameter.Themeansizewas5.5cm.Mammography showed nodular lesions and sonography demonstrated hypoechoic nodules. On histological examinationtherewasagranulomatousinflammatoryinfiltrateofepithelioidandgiantcells, withoutcaseationnecrosis,madeupoflymphocytes,plasmacellsandneutrophils.Microbiology investigationswerenegative.Lesioninvolvementwasprincipallylobulocentric.Surgicalexci- sionofthelesionswascombinedwithcorticosteroidtherapyintwelvecases,withnon-steroidal anti-inflammatorydrugsinanotherfourandwithantibiotictherapyinfourcases.Thepatients madegoodprogressintheshort-term.

Discussionandconclusion:IGM isarare entity. Itposes aproblem ofdifferentialdiagnosis becauseitclinicallyresemblesotherformsofmastitis.Thediagnosiscanbemadewithcertainty onhistologicalstudies.Thetreatmentismedicalalongsidesurgicalexcision.Theprognosisof thisdiseaseremainsfavourable.

∗Correspondingauthor.29,Lot.Abdelmoumen,RésidenceAlMokhtar,DerbGhallef,20340,Casablanca,Morocco.

E-mailaddress:mohcineb@yahoo.fr(H.Boufettal).

http://dx.doi.org/10.1016/j.diii.2012.04.028

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Idiopathic granulomatous mastitis is a benign lesion of the mammary gland characterised by the presence of non-infectious inﬂammatory breast lesions limited to the lobules.[1—3]. It is a rare nosologicalentity andfor this reasonitisnotwellknown.Itusuallyaffectsyoungwomen [4—6].Itsaetiologyandpathogenesisremainsasubjectfor debate [7—9]. It poses a problem of differential diagno- sisbecause itclinically resemblesother formsof mastitis and,mostimportantly,carcinomatousmastitis[10—12].The purposeof thisworkistostudyepidemiology,clinical and paraclinicalcharacteristics,pathologicalanatomyfeatures, treatmentandprogressofIGM,usingaseriesoftwentycases managedinourhospitaloveranine-yearperiodfrom2000 to2009.

Materials and methods

Wecarriedoutaretrospective studyofcasesofIGMman- agedover thelast9years,from2000to2009. Duringthis period,twentycases of idiopathicgranulomatous mastitis werediagnosedamongthe952patientswhoweretreated forbreastpathologies.Wethenanalysedtheepidemiolog- ical, clinical,radiological,andhistologicaldataaswellas informationon thetreatment andprogression ofpatients withthisclinicalentity.Epidemiological,clinical,treatment and progression data were speciﬁed for all of our cases.

The inclusion criteria were strictly based on histopathol- ogy: the only cases retained for this study were those withgranulomatous breast lesionslimited to the lobules.

Thesegranulomatouslesionsweremadeupofpolymorphous inﬂammatorycellsand/orepithelioidandgiantcellswithout caseationnecrosis, andnopathogenic agentswerefound, includingKoch’sbacillusonZiehl-Neelsenstainingandother pathogensonPASandGramstaining.

Results

Wecompiledalistofthe20casesofidiopathicgranuloma- tousmastitisoutof952patientswhoweretreatedforbreast pathologies,equating toa2%incidenceof IGM.Allof our patientswere female.Theyhad ameanage of 38.1years with extreme values of 19years and 66years (Table 1).

None of the patients presented a documented history of autoimmunedisease.Thetimetopresentationofidiopathic granulomatous mastitis in relation to the patient’s most recentbirthrangedfrom8daysto15years.Thetimescale

Table1 Distributionofgranulomatous mastitisby age ranges.

Ageranges(years) Numberof cases

Percentage(%)

19—29 2 10

30—39 11 55

40—49 5 25

50—59 1 5

60—66 1 5

Total 20 100

wasless thanﬁveyearsin12cases,which equatesto60%

ofcases.Lactationwasnotedin11patients(55%),withthe durationofbreastfeedingrangingfrom6monthsto7years.

Twelveofthepatientsweretakingoestrogen-progesterone contraception,withthetotaldurationrangingfromoneto 18years.Fourofourpatientswerepostmenopausal,equat- ingto20%ofcases. Themeanage ofthe menopausewas 45.5years, with extreme values of 43years and 48years.

None of the patients in our serieshad received hormone replacement therapy. Notable in the patients’ personal medicalhistories wassuspicionof abreastabscess in20%

ofthem andsurgerytothebreast foran adenofibroma in onepatient. A family history of breast cancer was found inone patient.None of the patients weresmokers. None of the patients had a history of autoimmune disease. No family history of IGM was found in our case series. The mean duration of symptoms of granulomatous mastitis in ourserieswas5monthswithextremevaluesof2weeksand 2years.Thecircumstancessurroundingdiscoveryofthedis- easeincluded a breastmass in 18patients (90% ofcases) (Table 2). The mean size of the lesion was 5.5cm with extremevaluesof2cmand18cmindiameter.Thepredom- inantareainvolvedwastheupperouterquadrant.Axiliary lymphadenopathies were present in five patients (25% of cases).The otherreasonsforconsultationbyorderoffre- quency were: a breast abscess in three patients (15% of cases),skinindurationintwopatients(10%),aninflamma- toryplaqueinonepatient(5%),alargeandinflamedbreast withnoevidentlesioninonepatient(5%ofcases)andiso- latedmastodyniainonepatient(5%ofcases)(Figs.1and2).

Twelve cases involved the left breast (60%), while eight involved the right breast (40%). Involvement was unilat- eralin19cases(95%ofcases)andinonecaseinvolvement wasbilateral.Findingsonmammographyincludedanopac- ityintwelvepatients(60%)(Figs.3and4),anasymmetric parenchymaldensityinthreepatients(15%),anopacitywith spiculatedmarginsinthreepatients(15%)(Fig.5),anopac- itywithindistinctmarginsandheterogeneousdensityinone patient(5%)(Fig.6)andcalciﬁcationsin onepatient(5%) (Table 3). Sonographyﬁndings were circumscribed images thatwere hypoechoic andhomogeneous in six cases,and hypoechoicandheterogeneousinanothersix (Fig.7).Fif- teenpatientsunderwentDopplersonography(75%ofcases), andthisshowedincreasedvascularisationofthelesionsand adjacentbreasttissue(Fig.8).

Microbiology investigations were carried out in eigh- teen patients, using breast discharge or breast biopsy

Table2 Theclinicalsignsrevealingidiopathicgranulo- matousmastitis.

Clinicalsigns Numberof cases

Percentage(%)

Nodule 10 50

Inﬂammatoryplaque 4 20

Abscess 3 15

Skininduration 2 10

Breastdischarge 1 5

Total 20 100

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Figure1. Enlargednodularleftbreastwithinﬂammatorysignsin a32-year-oldfemale.

Figure2. Enlargednodularandinﬂammatoryleftbreastina46- year-oldfemale.

Figure3. Bilateralmammogram inproﬁle: denseopacity with irregularmarginsintheleftdeepretroareolarregion.

Figure4. Bilateralmammograminproﬁle:deepleftretroareolar asymmetricdensity.

samples. These examinations were negative for Koch’s bacillus, Corynebacteria and all other bacteria. Cytologi- calinvestigationswerecarriedoutfor twopatientsinour series,andtheseshowedpolymorphousinflammatorycells withoutspecificity.Themainfeatureofthehistologyresults was the presence of an epithelioid and giant cell granuloma without caseationnecrosis, which wasseen inall of ourpatients,whileductalinvolvementsuchasocclusionor ectasiawasnotedinthreepatients,or15%,fatnecrosisin twopatients, or 10%, and collagen fibrosis in onepatient (Figs.9and10).Treatment forthisdiseaseis medicaland surgical.Sixteen patientsunderwentasurgical procedure, whichconsistedofwidesurgicalexcisionofthetumourin13 patients(75%ofcases),abscessdrainageandtumourectomy intwopatientsandabscessdrainageinonepatient.Allof ourpatientshadreceivedtreatmentwithdrugs,consisting ofaten-daycourseofantibiotictherapywithamoxillinor combinedamoxicillinandclavulanicacidin14patients(80%

ofcases).Non-steroidalanti-inflammatorydrugsaswellas antibiotics wereprescribed tosix patients (30% of cases) foranaveragedurationof7days,whilefivepatients(25%) wereprescribedcorticosteroidsasafirstline treatmentin the form of prednisone at a dose of 1mg/kg per day for 2monthswithgradualdosereduction.

Progressinboththeshortandmediumtermwasmarked by symptoms resolvingin all of ourpatients. These cases beingonaverage 4yearsold(between3and7years),full

Table3 Featuresonmammographyofidiopathicgran- ulomatousmastitis.

Mammographyfeatures Numberof cases

Percentage(%)

Opacitywithindistinct margins

13 65

Asymmetricparenchymal densities

3 15

Opacitywithspiculated margins

3 15

Breastcalciﬁcations 1 5

Total 20 100

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Figure5. Bilateralmammogram:massessmallerthanacentimetrewithirregularmarginsintherightretroareolarregion:obliqueview (a)andenlarged(b).

Figure6. Mammograminproﬁleoftheleftbreast(withlocalised compression):opacitywithirregularshapeandmarginsandhetero- geneousdensityintheleftsuperﬁcialretroareolarregion.

resolutionhadbeenseeninsixteenpatients(85%ofcases).

Fourcases hadarecurrence aftertreatment wasstopped andneededtorestartcorticosteroidtherapy.Dataconcern- ing the symptomatology of the patients in our series are summarisedinTables4and5.

Discussion

A number of terms have been used to designate granulomatous mastitis: lobular granulomatous mastitis, non-infectiousgranulomatousmastitis,post-partumorpost- lactation granulomatous mastitis [13—15]. These last two have not been adopted because the implications are not alwaysspeciﬁctothediseasegiventhelengthoftimethat canpassbetweengivingbirthorbreastfeedingandthedis- easedeveloping. Furthermore,useof theterm ‘‘lobular’’

maynotalwaysbeaccurategiventhatductallesionsmay bereportedinthisdisease[16—19].

The term granulomatous mastitis was introduced by Veyssiere et al. [3] in 1967 who were the ﬁrst team to

describeIGM.Fromtheviewpointofclinicalfeatures,histol- ogyandprogression,itwastrulyestablishedasanindividual entity by Kessler and Wolloch [20] in 1972. Very little is known to this day about its aetiology and pathogenicity.

Severalhypotheseshave been put forward toexplainthis secondaryinﬂammatoryreactiontomechanical,traumatic, hormonalormetabolicfactors.Theinitialcauseissuggested tobedamagetotheductalepitheliumthatcausesextrava- sationofglandularsecretionsintotheconnectivetissueof thelobulecreatinglocalinﬂammatorylesions[21—26].Oth- ers have also proposed immunological disorders [27—30].

Anautoimmuneprocesshasbeensuggestedinpatientswho haveundergonepregnancies,withthediseaseusuallyaris- ing in the 6years after the pregnancy [14]. The specific inflammatory involvement, identicalto that of cutaneous lesions,notablyinitshighquantitiesofmatureneutrophils andwithnoidentifiableinfectiousorganism,wouldseemto beanargumentforincorporatingthisentityintothecate- goryofhidradenitissuppurativaandconsequently intothe neutrophilicdermatoses,meaningthatthesystemicdimen- sion of this entity could be understood in this way [9].

Thepatient’sageatthetimeofdiagnosisvariesdepending ontheseries,rangingfrom17to83years[31—35,19].The meanagefordevelopingthediseasewas38yearsoldinour series,withextremevaluesrangingfrom19to66years.It mostcommonly affectswomenofreproductiveage,butit canalsobeseen in postmenopausalwomen[36—39]. This wasborne out by our series, in which four women were postmenopausal. In general, IGM develops in the 5years following the patient’s most recent birth [1,19,36,37]. In our series, 60% of patients presented the disease within 5yearspost-partum.The patientis usuallyinagoodclin- icalconditionandfeverisrarelyseen.Inflammatorysigns, whether systemic or localised to the breast, are a char- acteristicof IGM [12,33,36,40]. Inour series,six patients presentedlocalinflammatorysignsatthetimeoftheclin- icalexamination. Thenodule-or tumefaction-type lesions canhaveeitherdistinct orindistinct margins,andmaybe eitherhardorfirm[1,2,4—8,19].Inourseriesfivepatients presented hard lesionsand threeothers had firm lesions.

The size of the lesions is variable, on average between

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Figure7. Sonogram ofthe rightbreast: multipleoval masses withpolylobularmargins withaheterogeneous echostructure that is predominantlyhyperechoicwithenlargedaxiliarylymphnodes(aandb).

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Figure8. Bilateralbreastsonogram:multiplerightbreastmasseswithirregularmarginsandahighlyhypoechoicechostructurewithan irregularhyperechoichalo.

Figure9. Histologicalfindingsshowingidiopathicgranulomatous mastitis characterised by epithelioid and giant cell granulomas withinapolymorphousinflammatoryinfiltrate(enlarged×10).

Figure10. Epithelioidandgiantcellgranulomaseenontheopti- calmicroscope(yellowarrow)madeupofgiantcells(bluearrow) andepithelioidcells(whitearrow)enlarged×40.

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Table4 Summarytableoftheepidemiologicalandclinicaldataaboutourpatients.

Case Age(years) History Reasonforconsultation

anddurationofsymptoms

Clinicalexamination

Case1 19 Nomedicalhistory Self-palpationofaleft breastnodule1month priortoadmission

Nodulemeasuring3/2cmof theUOQoftheleftbreast Case2 25 Breastfeedingfor2years

Oraloestrogen-progesterone contraceptionfor3years

One-yearhistoryofbreast dischargeandskin indurationfor1month

NoduleintheUOQoftheleft breast

Adjacentskininduration Case3 52 Breastfeedingfor4years

Oraloestrogen-progesterone contraceptionfor7years Adenoﬁbromaattheageof 25

Two-weekhistoryofan inﬂammatoryplaqueof therightbreast

Inﬂammatoryplaqueofthe UOQoftherightbreast Ipsilateralaxiliarylymph nodepathologyaroundone centimetreinsize

Case4 45 Breastfeedingfor3years Oraloestrogen-progesterone contraceptionfor5years Breastabscessdrained 5yearspreviously

Four-monthhistoryof breastabscessrefractory totreatment

Nodulemeasuring8/5cmof theUOQoftheleftbreast withadjacentsignsof inﬂammation

Case5 47 Nomedicalhistory One-yearhistoryofleft breastabscessresistantto antibiotictreatment

Nodulemeasuring10/7cmof theUOQoftheleftbreast withinﬂammatorysigns Case6 66 Breastfeedingfor7years Two-weekhistoryof

progressivelyenlarging rightbreast

Enlargedinﬂammatorybreast

Case7 35 Oraloestrogen-progesterone contraceptionfor3years

Six-monthhistoryofright breastnodule

6/5cmmoduleoftheUOQof rightbreast

Case8 36 Nomedicalhistory One-yearhistoryofright breastnodule

8/5cmnoduleoftheUOQof rightbreast

Case9 28 Nomedicalhistory Six-monthhistoryofleft breastnodulewith mastodynia

5/3cmnoduleoverlapping bothOQoftheleftbreast Case10 23 Nomedicalhistory One-monthhistoryofleft

breastnodule

3/2cmnoduleoftheUOQof theleftbreast

Case11 47 Oraloestrogen-progesterone contraceptionfor6years Breastfeedingfor4years

Two-yearhistoryofleft breastnodule

4/3cmnoduleoftheUOQof theleftbreastwitha1cm axiliarylymphadenopathy Case12 44 Oraloestrogen-progesterone

contraceptionfor3years Breastfeedingfor4years

Five-monthhistoryofright breastnodule

10/5cmnoduleoverlapping bothOQoftherightbreast withadjacentskininduration Case13 35 Breastfeedingfor1year

Oraloestrogen-progesterone contraceptionfor4years Breastabscess4years previously

One-yearhistoryofleft breastnodule

Case14 52 Breastfeedingfor7years Oraloestrogen-progesterone contraceptionfor3years

Two-yearhistoryofleft breastnodule

Case15 34 Nomedicalhistory Five-monthhistoryofright breastnodule

6/4cmnoduleoftheUOQof therightbreast

Six-monthhistoryofleft breastnodule

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Table4(Continued)

Case Age(years) History Reasonforconsultation

anddurationofsymptoms

Clinicalexamination

Six-monthhistoryofleft breastnodulewith increaseinbreastsize

18cmtumourtakingthe entireleftbreast Case18 38 Breastfeedingfor2years

Oralcontraceptionfor3years

One-yearhistoryofright breastnodule

4/3cmnoduleoftheUOQof therightbreast

Case19 35 Oralcontraceptionfor1year Rightbreastnodule 5/3cmnoduleoftheUOQof therightbreast

Case20 38 Breastabscess4years previously

Six-monthhistoryofright breastnodule

6/3cminﬂammatoryplaque overlayingtheUOQofthe rightbreast

OUQ:upperouterquadrant;OQ:outerquadrants.

5 and 8cm. The sizes reported in the literature usually range from 0.5 to 15cm [10,18,20]. In our patients, the mean sizeof the lesionswas 6.5cm andranged from 2.5 to15cmindiameter.Nippleretractionandsaggingarenot reported often in the literature but this is a further fac- tor for exploring differential diagnosis from a malignant process[34].Nippleretractionandsaggingwereobserved in one patient. One explanation proposed for this nipple retraction is the spread of fibrosis in later-stage disease [12,33,36].IGMisoftenunilateral,butafewcasesofbilat- eralinvolvementhave beenreported[1,4,18].Oneof our patientshadbilateralinvolvement.Secondaryaxiliarylym- phadenopathiesmayoccurandtheyhavebeenreportedin between40and60%ofcasesinanumberofseries[1,2,4].In ourseries,axiliarylymphadenopathieswerefoundinthree cases(37.5%).Mammographycarriedoutattheinflamma- tory stage showed an overall increase in breast density associated with skin thickening. Findings could include a single,homogeneousnodularopacitywithdistinctmargins ordisorganisedstraightlinesproducinganimageofdiverg- ing tracts [19,23—29,38,41]. Sonography is carried out as an adjunct to mammography. It often demonstrates non- specificimagesinIGMthatcanalsobesuggestiveofmalign disease.Thefeaturesmostcommonlydescribedaresingle or multiple irregular hypoechoic masses that are some- timesheterogeneous[15,23—27].Theseimageswerefound in half of the cases in the seriesreviewed by Balaabidia [1] and by Engin [42]. The areas surrounding the lesion often have ahyperechoic structure [15,26].In ourseries, ten patients (55.6%) presented this pattern. Hypoechoic tubularlesions,eitherseparatedorcontiguous,werefound in 66% of the cases described by Han [44] and in half of the cases reported by Lee [41]. According to Engin [42], if multiple heterogeneous images with relatively circum- scribedmarginsandatubularconfigurationcombinedwith a large hypoechoicmass appear onsonography, thismust prompt the clinician to suspect granulomatous mastitis.

Anotherfeatureobservedwasabsorptionoftheultrasound beam in the posterior part of thelesions, asreportedby Van Ongeval [7] and in the series reviewed by Alper [21]

in 23%of patients. Other features thatmay befound are parenchymal distortionthat maybeextensive andimages

ofanabscessthatmayhaveasecondaryfistula.Incasesin whichabscesses havedeveloped, sonography canbe used toassesslesion size andtobetter understand whichtype of drainage(puncture or surgical debridement) shouldbe used [15,26,44]. Doppler sonography was carried out by Engin[42]insevenpatientsandshowedanincreaseinarte- rialandvenousvascularisationaroundthelesionsin75%of cases.Theseobservations,however,remainnon-specificto thedisease,andindeedthesamefeaturescanbefoundin otherbreastpathologies,particularlythoseinvolvingmalig- nancy [16,42,43]. Doppler sonography was carried out in 14patientsinourseries.Itrevealedincreasedvascularisa- tionofthelesionsandtheadjacentbreasttissue.Recently, numerous studies have reported using MRI to investigate breastpathologies[23,24,27].Thistechniquedemonstrates lesionssuspiciousformalignityintheformofirregulartissu- larmasseswithanabnormalcontrastuptakeafterinjection of gadolinium chelates. In the series reviewed by Alper [21], MRI demonstrated a homogeneous mass with irregular margins in two patients, parenchymal distortion in threepatientsandparenchymalasymmetryin onepatient althoughtwoofthesesixpatientshadpresentedanormal mammogram. One patient with recurrent granulomatous mastitiswhounderwentMRIpresentedimagingofalesion thathad the appearance of an abscess. This examination remainstotallynon-specific,sincethesamefeaturescanbe seen in breast carcinoma. Some authors believe that MRI would be useful to evaluate the extent and reduction of lesionsovertime[23,27].Imagingcanconfirmmastitisbut onlyhistology enablesa diagnosis tobe made[40,43,44].

Abnormal blood results are often reported in patients withIGM. This means thatinvestigations ofaetiology and immunology include C3, C4, CH50, antinuclear antibod- ies,anti-DNA,anti-streptolysincountandsyphilisserology [12,33,36]. These investigations may show abnormalities when IGM is present in the context of systemic disease.

Microbiologyinvestigationslookingforanon-speciﬁcinfec- tioninpatientswhohavedevelopedabscessesalmostalways have negative results [27,33]. Corynebacterium accolens wasisolated asa human pathogen froma breast abscess inapatientpresentinggranulomatousmastitis[45—47].In ourcases,microbiology investigations,includingthosefor

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Table5 Summarytableoftheradiological,histologicalandtreatmentdataofourpatients.

Case Mammogram Sonogram Histology Treatmentandprogress

Case1 Increasedopacity Heterogeneous hypoechoicimage

EGCGwithoutCN Collagenﬁbrosis

NSAID Antibiotics Wideexcision Case2 Increasedopacity Homogeneous

hypoechoicimage

EGCGwithoutCN Ductectasia

Abscessdrainageandtumourectomy Antibiotics

NSAID Case3 Asymmetricdensity Heterogeneous

hypoechoicimage

EGCGwithoutCN Antibiotics Wideexcision Case4 Spiculatedopacity Heterogeneous

hypoechoicimage

EGCGwithoutCN Ductectasia

Abscessdrainage Antibiotics NSAID

Case5 Increasedopacity EGCGwithoutCN

Fatnecrosis

Abscessdrainageandtumourectomy Antibiotics

Corticosteroids Case6 Asymmetricdensity

withmicrocalciﬁcations

EGCGwithoutCN Antibiotics Corticosteroids Case7 Increasedopacity Heterogeneous

hypoechoicimage

EGCGwithoutCN Antibiotics Tumourectomy

Case8 Increasedopacity EGCGwithoutCN Antibiotics

Wideexcision Case9 Increasedopacity Heterogeneous

hypoechoicimage

EGCGwithoutCN Fatnecrosis

Antibiotics Wideexcision Case

10

Increasedopacity Homogeneous hypoechoicimage

EGCGwithoutCN Antibiotics Wideexcision Case

11

Asymmetricdensity witharchitectural distortion

Heterogeneous hypoechoicimage

EGCGwithoutCN Antibiotics Wideexcision NSAID Case

12

Spiculatedopacity EGCGwithoutCN Antibiotics

Wideexcision NSAID Case

13

Increasedopacity Heterogeneous hypoechoicimage

EGCGwithoutCN Antibiotics Wideexcision Case

14

EGCGwithoutCN Ductobstruction

Antibiotics Wideexcision NSAID Case

15

Increasedopacity EGCGwithoutCN Antibiotics

Wideexcision NSAID Case

16

Wideexcision Case

17

Spiculatedopacity Homogeneous hypoechoicimage

EGCGwithoutCN Antibiotics Corticosteroids Case

18

Wideexcision Case

19

Wideexcision Case

20

EGCGwithoutCN Antibiotics Corticosteroids EGCG:epithelioidandgiantcellgranuloma;CN:caseationnecrosis;NSAID:non-steroidalanti-inﬂammatorydrugs.

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Koch’s bacillus and Corynebacteria, were negative. Fine needleaspirationisavaluabletechnique,asitrevealsLang- hansorMüllerepithelioidcells.Inadditiontothese,other cells found in varying proportions aremacrophages,foam cells, lymphocytes and plasma cells. Epithelial cells may be present and sometimes show slightly atypical regen- eration that could be mistaken for malignity [8,20,34].

Cytologyspecimensexaminedwithspeciﬁcstains(PAS,Gro- cott etc.) toinvestigate pathogenic agents may be used.

Microbiology of the specimen enables an organism to be investigated.Fineneedle aspirationdoes have limitations concerningtechnicalissuesandproblemsofinterpretation.

Biopsy,however,allowsIGMtobediagnosedwithcertainty [8,20,33—35,19,36—38].Pathologicalanatomystudiesback upthisdiagnosticcertainty.Lesions maybeconfluentand this canmean that theyjutoutside of the breastlobule, whichexplainstheappearanceofmultipleconfluentnodules or hypoechoic tubular nodules on sonography. A hypere- choicperipheralhalocorrespondstothewallofsurrounding fibrosisthatborders thegranulomas[1].Thisfindingleads to a problem of differential diagnosis with tuberculosis, especiallyincountrieswhereitisendemic,butthepredom- inanceofneutrophilsandtheabsenceofcaseationnecrosis isanargumentinfavourofgranulomatousmastitis[8—10].

IGMclinicallyposesaproblemofdifferentialdiagnosiswith carcinomatousmastitisduetonippleretraction,hardness, ﬁxed position, orange peel skin and lymphadenopathies.

ThediagnosisofIGMmayequallybeproposedinbacterial, parasiticor mycoticinfections ofthebreast,aswell asin non-infectiousgranulomatouslesions(lipophagicgranuloma or fat necrosis, sarcoidosis, plasma cell mastitis, lympho- cytic mastitis etc.). These different pathologies can be distinguishedbymeansofhistologicalinvestigations[34,36].

Treatmentisessentiallysurgical,consistingofwideexci- sionof thelesion preceded bycorticosteroidtherapy, the aimofwhichistoshrinkandlimitthelesions[12,33,48—50].

Corticosteroidshaveonlybeenusedoccasionallyandifthey have shown some efﬁcacy, they must not be used with- outbeingcombinedwithantibiotics[50,51].Ahighfailure rate for corticosteroid treatments is reported in the literature and the incidence of recurrence varies between 16 and 50% of cases depending on the series [12,33,36].

Thispresentsanopeningforother treatmentalternatives, namelymethotrexateandantibiotics[9—11].Casesofrecur- renceorrelapsehavebeenreportedseveralyearsafterthe patienthasrecovered[18,26,29].Fourpatientsinourseries hada recurrenceafterstoppingtreatment andneeded to restartcorticosteroids;oneofthesepatientsrelapsedthree times.However, aresolution of symptomswasseen in all cases.

Closeandregularmonitoringwillaimtodetect disease recurrence at the earliest opportunity in order to avoid repeatedsurgicalinterventions. Monitoringonly,withnei- thersurgerynorcorticosteroidtherapy,hasbeenreported bysomeauthorswithsignsresolvingandnodiseaserecur- rencein50%ofcases[1].

Conclusion

Granulomatousmastitis is rareand presentsa problemof differential diagnosis in terms of imaging features, with

particularreference tobreast cancer. It should bestated thatthemainvalueofsonographyistodemonstrateabnor- malitiesthatmaybesuitableforbiopsy,whichthenenables adiagnosistobemade.Theclinicalcontext,thevariability ofimagingfeaturesonexaminationsdoneatcloseintervals andprincipallyhistologicalexaminationallowthediagnosis tobedrawnoutandthetreatmenttobeadapted,avoiding disﬁguringsurgery.The treatment of choiceis widesurgi- cal excision of the lesions, combined with corticosteroid therapy in order to avoid relapse. Regular monitoring is indispensableinordertodetectdiseaserecurrenceatthe earliestopportunityandthereforetoavoidfurthersurgical interventions.Theprognosisisgenerallygood.Nonetheless, patients maytake some time tomake good progress and experiencediseaserecurrencewithrepeatedinterventions andthiscanleadtodisﬁguringsequelae.

Disclosure of interest

Theauthorsdeclarethattheyhavenoconﬂictsofinterest concerningthisarticle.

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