Journal of Cancer and Tumor lnternational2(4): 1 50-l 54, 201 S, Article no.JCTt.2}t S.Ot 6

tSSN:2454-7360

SCIENCEDOM AIN inte rnationatwww. sci e nced o mai n. o rg

Myofibroblastoma of Axillary soft Tissue in a childShubhangiV. Agale1., Bhushan M. Warpel, Geeta Kumaril

and Arvind G. VatandlI Department of Pathotogy, Grant Govt Medicat college, Mumbai-40000g, tndia.

Auth ors' co ntrib uti o n sThis work was carried out in cotlaboration between all authors. Author svA diagnosed the case,designed the study, wrote and rectified the manuscript drart triti p"iirio)n. Author BMW managedthe literature searches and wrote the first draft of tie iinr'iriipiiiii-iJtp ot authors GK and AGVreviewed the manuscript. Atl authors nave relai aii ,iiireo tne rinai iinis'irlpi

Article lnformation

DOI: 1 0.9734/JCTti2O1 5/206s3(1) Nicole Riddle, Department of pathorogy, The university of rexas Hearth science center (urffi

USA.

.- (1)Anonymous, Tzaneio General Hospital of pir""uffm.(s)enonvmou,,(?ofyT'.':?:$!1'fi:,:lJ#i!;H:ilil_il,:lf:1,"t*t,3:;*:

comprete pee ; review rlll"t, ifl:1,1}#lS;"Hl;'i.ll,n,,T.xl,:..i;ii8l1

Received 1"t August 201iAccepted 21", August 20li

Published tt' September 20tS

scrrcmotall :

ABSTRACT

cells showins a variabre myoribrobrastic aitreieniiaiion "t

*oipriorogi;; ffiffi;::,ffi#:$T';ultrastructurat tevers. MFB. is commonry seen inadu.rt ilililfro;;Yii'"g. rung" of 36_67 years.

J}I"*T,T,.:'"1""'y-:i',:".,:.11?g:Ii:-pgnir.qa1,91 *it1 tei,,;ilnq.nd;;ts berow the ase or 21

f;:r;r.*" reporr mvofibrobiasroma in' a youngest femare ir,ira *ni.n-rffi;[i'; ffidffii

Presentation of case: A four-year-old lemale child.with history of tender left axillary swelling sinceone year' The swelling was initially small and gradually in;ild' ;-pi"r"nt 8 x 6 cm size. Thelump was oval, firm to hard, mobild, tender witliclear margins and smooth surface. MRI studies ofthe left shoulder and thorax revealed enhancing neoplastid mass in tnelett axilla inseparable fromthe subscapularis muscre measuring I x 5 x 6 dm. eios-sry tn" tril,;;;;sured 8 x 5 x 6 cm, wasX,"ll.li?l1ilj:b:j,:11,p.11i1|I_:ry1p:yrared,with a;ridl ;;i;i;-;hii;;, tan appearance. rhe

flil":Jfl!robr

overall f eatures f avou red the diignosis or ryttiU,roUru.ioii"iDiscussion: Myofibroblastoma ls a benioi mesenehvmrt

' Correspondi ng author : E mai I : shubh ag ale@ hotmait.com ;

Agale et al.; JCT\, p(4): $e|54, 20tS; Atticle no.JCTl.ZOtS.ot6

ABBREVTATTONS inseparabre from the subscapuraris muscre

MFB-Myofibrobtastoma. measuring I x 5 x 6 cm (.Figq, io ano rc). i-hepectoralis major and pectoralis minor muscles

1. rNrRoDUcroN yf;"I'j,n:l"X{.lilJT:y;.':? ;il':ffi HX{a

and ribs were not involved by the tumourI\rlrra{irrr^kt^^r^-- !- -,Myofibroblastoma is a benign, prototypic tumourot the mammary stromj and wis initiallvdescribed- as a typical tumour occurring in thebreast of adult males [1-g]. MFB w:as lirstdescribed in 1987 by Waigotz et at. [1,+l ui iorstncltve benign mesenchymal tumor of thebreast. The tumour is comprised ot spinOte cetiswith a variable fibromyobtistic dif{erbntiation- iimorphologic, immunohistochemical ,nO'ultrastruclural levels.

swelling was initially imall and 'oraduallv

increased to present g x 6 cm size. T=he lum6was oval, firm to hard, mobile, tender with clearmargins and smooth surface. The overlying skinwas normal without any prominent ieiis orfixation to lump. There was no transmittedpulsation or bruit on auscultation. There *", nog,1tn-aUle lymphadenopathy of cervlcal, supra-clavicular, axillary and internal mammaiy groupof lymph nodes. The general anO syslemi'cexaminations were unremarkable.

Extra-mammary location for this tumour is vervrare. The extra-mammary sites are locatedmainly along the embryonic milk line [2,4,S,6].

Till date only 20 cases of extra-mammarv MFBhave. been reported I4]. According 6 oriKnowteoge, our case is the youngest reportedzt case or extra-mammary myofibroblastoma.

2. CASE PRESENTATIONrvr\ .. Fig. 1. A) 1a- X_ray chest revealed a softttssue tumour in left axilla and both lungs areA four-year-old femate ctritd with normat ,nr"rrr[rui"; ;i1; & 1c- MBt studies of thedevelopmental milestones ya: brought bt lefi shouldeiind il.rora revealed enhancingparents to Paediatric surgery. Unit wlth nistory ot neoplastic ,nr"s in it

" i"fi;;il;;;ffi;;tender left axillary sweiling since one year. the a x s i 6;;-' - "'.vsee,"r

X-ray chest revealed it as a soft tissue tumor inleft axilla.measuring 8 x 5 x 6 cm (Fig.fa1. MHistudies of the lett shoulder and thorai revealedenhancing neoplastic mass in the left aiiili

f]9. Z. A) 2a. Grossty welt-circumscribed,partly encapsulated tumour measured g x 5 x

6 cm; B) 2b- Cut surlace revealed solid,- --

grayish-white to tan, firm tumor with mvxojdareas without hemorrhage and necroiis

EHty.xt:iEngtish titerature onrv six ."'"1 r,u,. o;"; ';J,.i;l",.,Y I il,"il"rlJ,J l",: ;iTX;t

"'*'"?,.IIi: :ll:1,::: f "";1 -g * : :* ::i :J lr:il ",'

oiJs i, f, i in f, ri i ii x i *" r r i n sconclusion: Extra-mammarv mvoribrobrastoma-il ;';"il';i1i;:].il:'il;;'llfli". in paediatricpopulation below the age of tive years.

Keywo rd s : B e n ig n m e se'nchy m a ! tu m ou r ; myof i bro b rasto m a ( M FB) ; soft-ti s s u e.

Fig.2b

151

FNAC from the swelling was suggestive ofspindle cell lesion. lntra-operatively-tie tumourwas well-circumscribed without infiltiation into theadjacent soft tissue. The excised specimen waisent for histopathology. Grossly the tumourmeasured 8 x 5 x 6 cm, weighed 400 grams andwas well-circumscribed and oartiallvencapsulated (Fig. 2a). The cut surface waisolid, grayish-white to tan, firm with myxoid areasbut no necrosis and haemorrhage (Fig. Zb).

Agale et al.; JCTI,2(4): tS0-154, z7tS; Atticte no.JCTl.2Otg.Ot6

staining showed diffuse positivity of the spindledtumour cells lor CD34 and Desmin (Figs. 4a and4b), however S-100 and cytokeritin werenegative. The overall featurei favoured thediagnosis. of myolibroblastoma. Our patientrecovered well after surgery withoui anycomplications. No recurrence oi tne lumor wasseen after complete surgical excision on follow-up duration of five months.

Fig. 3. A) 3a- Microphotograph showed an. well-c.ircumscribed, partly encapsulated,hypocellular, vaguely noduiar myxoid tumour

with foci ol adipose tissue. No areas ofhemorrhage and necrosis were seen. (H & E.

x a0); B) 3b-Tumour cells were embedded in amyxoid stroma and were plump to spindle to

- stellate with plump ovoid pale nuclei.!cca9]91al.nuctear grooving was seen. (H &E, x +00); C) 3c-Tumour showed large areas

ot c-artilaginous differentiation. (H & E, x 100);D) 3d-The stroma was characterized bv "

coarse collagen lormation with occasional- .giant cells. (H & E, x 1000); E) 3e- Few

epithelioid cells were also seen in the stroma.(H&E,x1000)

On..microscopy, it was a well-circumscribed,partially encapsulated, hypoceilular, vaouelvnodular myxoid tumour with large

"r.ai oicartilage and foci of adipose tisiue (Fig. 3aabove). The tumour cells were emUeOdeci'in amyxoid stroma and were plurnp to spindle tostellate with plump ovoid pate nuclei withoccasional nuclear grooving (Fig. 3b). Thestroma was characterized by coarse collagenformation with chondroid difieientiation (Fio. 5c).Also occasional giant cells and epitnetibij ceitswere seen (Figs. Ad and Se). Areas ofhemorrhage and necrosis were absent. lmmuno_

Fig. a. A) 4a- lmmuno-staining showed dilfusepositivity ol the spindled tumour cells for

.- CD34; B)4b- lmmuno-staining showeddiffuse positivity of the spindledlumour cells

tor Desmin

3. DISCUSSION

Myofibroblastoma is a benign mesenchymaltumour_commonly involving breast in elilerlymales. The extra-mammary location is very rarefor this tumour [4].

The literature review shows that extra_mammaryMFB is rare with few reported cases and hasmale predilection and median age of 53 years(36-67 years). In English literaturJ only six caseshave been reported below 21 years 6t age inOmost were teenagers. The prior younoestreported case was in a nine-year-old giri witfitettgroin swelling [7]. The extra-mamirary MFBcases.reported below 21 years of age ar-e givenin Table 1.

To our knowledge, there is no MFB caseoccurring below five years of age. Thus this istne youngest reported MFB case.

It has been extensively studied in literature thatthe location of extra-mammary MFB is mainiyalong the embryonic milk-line extendino frommid-axilta to medial groin [2,4,6]. Nevert-helesslew cases have been reported outside milk-linesites like buttock, mid-back, para-testicuiarregion, senrinal vesicle, vulva, vaginal wail,popliteal fossa, thigh, liver and lead_neckregions [3].

152

ATqnS.the extra-mammary location of MFB, thepelvic sites are more in number than extra_pelvicsites. The pelvic sites are five cases in inouinatarea, three cases each in vulva and vagini, twocases each in para-testicular region anO Outioct<,one case each in seminal vesicle, peri-analregion and supra-pubic area. None nave Ueenreported in axillary soft tissue [4]. The six casesreported outside the pelvis are mandibular areapopliteal fossa, abdominal wall, fiver, Uacf, anf,thigh. There is only one MFB case in axitta wnicnnas been reported by Mackay et al. [2].

Most of the tumours are diagnosed incidentallvand are of small size. The iumor size ,rnguifrom 2-'13 cm with median size of S.S cm12t4,5,61.

Recenfly Abdut-Ghafar et at. in zOiS t+fhave reported a largest myofibroblastima of b4cm. in medial aspect of thigh in a SO_year_oldmale.

Grossly this tumor is well circumscribed, andpartly encapsulated. Cut sudace is orayish whit"lpink with whirling or nodular appeaiance with noevidence ol necrosis, hemorrhaoe.Microscopically mammary MFB shows variiLtvsized fascicles of spindle to oval celts embeOOilin -hyalinised and collagenous stroma withvariable intra-lesional fatly component. fewcases have shown cartilaginous and smoothmuscle diff erentiation t1i. ln our .ar",macroscopic and microscopic features aresimilar to that of mammaiy-type MFB, .bui

Agale et al.; JcTl,2(4): 150-154, 2015; Atticte no.JcTt.2ol5.o16

characteristically cartilaginous dlfferentiation waspresent with occasional giant cells and epitnetoiOcells. Other MFB variints wnicn nave-- Oeen

l-n:il.d inglr g.? c.oilageno us/ fibrous, "pitnJo

iO,ceilutar and inf iltrative types [1 ,2,4].

So far known, all the 20 cases of extra-mammarymyofibroblastoma show both ,a.ro anOmicroscopic features similar to ,irr"iVmyofibroblastoma [a], except on" ..i" *iti,mlxect pattern of angiomyofibroblastoma andmammary-type myofibroblastoma of soft tissueU l. un immunohistochemistry, MFB showspositivity for CD34 and Desmin (Z,C,S,Sl.

The. differential diagnosis of this tumor includesoentgn lesions such as spindle cell lioomaang iof ibroma, angiomyofibro5tastora,-sott issu?penneuroma and nodular fasciitis. Few malionanttumours which should be taXen 'inioconsideration include spindle cett tipoiircoma.lov'r grade malignant Ieripheral nurr"-rn.riltumor and dermatof ibrosarcoma protruberans[2,4]. As these tumours are benigri, id;;;;;excellent prognosis. No recurrence or metbstasisrls seel after-surgical excision i" th; ;;p;;;cases [2,4,5,6].

Awareness about this tumour occurring inpaediatric population amongst paediatric

!!lgu?.nl: oncotogists and pathotogisrs isessential for the management.

Report

Current Case 2015

Santos et al. 2012t8I

Female 12

2012- Female 9

ttalionTender 8x5x

6cmaxillary mass

Painless S cmenlargement oflymph nodePainless mass 4 cm

Asymptomatic 3.5 cm

Painless mass 5 cm

Painless mass B cm

Tumoursize

Left axillarysoft tissueMASS

4.

2.

5.

b.

Chami et al.T4

Magro et al.tsILee et at. [6]

Female 15

Female 12

Female 16

Lymph nodeof inguinalregionLeft groin -lymph nodemassOral cavity

Anteriorchest wallMASSVulva

2012

2005

Montella et al. ZOO4

t10lQublan et al. ZOO?

Table 1' Reported cases or extramammary myoribrobrastoma berow 21 years or age

153

4. CONCLUSION

Extra-mammary myofibroblastoma is a rare entityand is uncommon in paediatric population belorirthe age of five years.

CONSENT

All authors declare that written informed consentwas.obtained from the patient (or other approvedparties) lor publication of thls case report andaccompanying images.

ETHICAL APPROVAL

It is not applicable.

COMPETING INTERESTS

Authors have declared that no competinginterests exist.

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3. Arsenovic N, Abdulla KE, Shamim KS.Mammary-type myofibroblastoma of softtissue. lndian J pathol Microbiol. 20.1 1;54:391-3.

Agale et al.; JcTl,2(4): 150-154, 2015; Afticte no.JcTt.zll5.o16

4. Abdut-Ghafar J, Din NU, Ahmad Z, BiilingsSD. Mammary-typemyofibrobtaitorJttthe right thigh: A case report and review ofthe literature. J Med Case Rep. 2015;9:126.

5. McMenamin ME, Fletcher CD. Mammary_type myofibroblastoma of soft tissue: Atumor closely related to spindle cell lipoma.Am J Surg pathol. 2OO1;ZS:1022-9. '

6. Lee H, Lee OJ, Cho KJ, Flo Hy. EpithetioidMyofibroblastoma of Mammary-1yps

-;r.l

Chest Wall - A Case Report. The KoreanJournat of pathotogy. 2005;S9:180_0.

7. Chami R, Ertresvaig K, Azzie G, ihornerPS, Myofibroblastoma: report ol a ,ai"entity in the pediatric popirlation. pediatrDev Pathot. 201 2;1 5:49'9_SOO.8. Santos Ml, Salgado C. lntranodalmyofibroblastoma: A case report. Ci,Pediatr. Z0 1 Z;ZSi1 17 -9.

9. Magro G, Caltabiano R, Kacerovskd D,Vecchio GM, Kazakov D, Michai M.Vulvovaginal myof ibroblastoma: expandingthe morphological and immuhofristo_chemicat spectrum. A ctinicopatholoitstudy of 10 cases. Hum' patn-ot.2012;40:243-53.

10. Montella F, Giana M, Vigone A, Surico D,Surico N. Angiomyofibroblastoma of thlvulva: Report of a case. Eur J GynaecolOncol. 2004;25:253-4.

1 1. Qublan HS, Fayyad LM, Al-Ghoweri- AS. Angiomyofibroblastoma of the vulvaoccurring in a teenaged girl. J ObstetG yn aecol. Z00Z ;22:562-9.

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peer-review history:The peer reuiew history for this paper can be accessed here:

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