British Journal of Medicine & Medical Research11(2): t-6, 2016, Article no.BJMMR.206J5

tSSN:2251-0614

SCIENCEDOM AIN internationatwww. s c i e n c ed o m ai n. o rg

_ solitary Fibrous Tumor of Head & Neck Region:

Problems in Diagnosis Due to ',Borderline,, Feat-ures

shubhangiv. Agarel., vidya c. Arotel, Akash Jogi1, Aravind G. Varandland Akshay K. Tiwaril

l Department of Pathotogy, Grant Govt. Medical college, Mumbai-40000g, tndia.

Auth ors' co ntrib uti onsThis work was carried out in collaboration between all authors. Author svA diagnosed the case,desisned the studv and wrote rirst drart ana rectiii th" ;;;;;;;;i;;;i;';, its perrection. Author vcAhelped to write first draft.,Author AJ managed the titeratutiiirZiiiri' iitn hep of authors AKT andAGV reviewed the manuscript- Alt authors naue ,eii ira-approvea the final manuscript.

Article lnformation

DOt: 1 0.9734/BJMM R/201 6/20695(1)'Franciszek Burdan, Experimental reratology unit, Human Analomy Department, Medical University of Lublin, *##o

Radiotogy Department, St. John,s Can""r Centei,iotanO.

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comprerepeerreviewr,."l;]f*tJi,:55L$*:,X"."t1:l?.,:;,_,n:lil.I,?iru

Beceived.1't August 201 5Accepted 31"' August 2015

Published 21st September p01S

ABSTRACT

:ToreportSFm^'r'r-' rvrePUrrorr rrr'1 +oyearsmalewnonadalobulatedtumorinnecktorlongestdlEiffi-18 years with borderline histomorphology.presentation;ic;;;;fi;;"..1"jffil's;d

is of a forty{ive year ord rndian man who presentedwith painless mass in right side of neck lor the duration oi ial"uo wii['rapio entargement inlast one year.Discussion: solitary fibrous tumor is an unusual..spindle cell neoplasm arising from primitivemesenchymal cell with di;stinct "patternles.ss pattern" dn nistomorpnJ6gy. rn" commonest site forthis tumor is pleura but in recent years it has been described in ,rr"i,j* analomic sites and islngwn t9 involve anyregion of th^e-qgdy. The extra- pleuralsites inctuoe extremities, pelvis, headand neck and urogenital region. sFT oi Head and.N'err rcgionl" r"re irio usuatty involves deepsoft tissues. M-orphologicaily. SFT resembres many benign u"nJ rurig;"nirott tirrr" tumors.

:,i::':T,j:".:Il'.jjT:;ly,T:ll., T:l_,es;;;J h;; t.o;;;;"#.,tiarLa meticurousry rromother common and rare solt tissue trro".-dior" forow up i. "r..nti"i"tt"l;;ffii#J:,1rffi:lexcision in cases with ,,borderline,,

histonorphology.

'Conesponding author: Email: shubhagale@hotmail.com;

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Agale et al.; BJMMR, 11(2): t-6,2016; Articte no.BJMMR.2o6s5

Keywords: Solitary fibrous tumor; mesenchymal tumor; head and neck region.

ABBREVIATIONS

SFT- Solitary fibrous tumor.H &E- Hematoxylin and eosin.

l.INTRODUCTION

Solitary fibrous tumor is a rare, mesenchvmaltumor affecting mainly middle aged adults withno sex predominance however can occur inyounger patients [1 -S].

This spindle cell neoplasm usually arises in thepleura but is known to involve extia-pleural sitesin.. 50-70."h.of cases [1,2]. These extra_fleurilsites include extremities, pelvis, head and neckand urogenital region t1-4].About 40% ofexlrapleural SFTs are located in subcutanecustissue while 60o/o ?ta located in the deep softtiss.ues of extremities, abdominal cavity,' retioperitoneum and head neck region [6].

R.ec-fy Vogel et al. [7] have reported 28 casesof SFTs at different sites (boih thoracic andextra-- thoracic) in which they have describedonly 2 cases of SFTs in head and neck regionwith one case in the neck region. ln head

"and

neck the commonest site is.oial cavity followedby orbit, nose and paranasal sinuses f4l.Subcutaneous tissue of neck is an unusuat iit!for this tumor.

Estimated 5-20/" ot thoracic solitary fibroustumors may have malignant features butmalignant extrathoracic tumors arc rare.However the clinical behavior of extra-thoracicSFT is unpredictabte [3,S].

Local examination revealed large, ovoid,lobulated sweiling ot 10.5X8.5 cm (Fj-$ ral. inLoverlying skin was stretched witn toiat'utceiation.

CT scan revealed well defined lobulated lesionshowing enhancement in subcutaneous tissue onright sid.e ot neck (Fig.1b and 1c). USC necf<revealed a. large well defined, .rootntymarginated, heteroechoic lobulated ,as. in itLright side of neck.

Due to. suCden enlargement of the lesion whichwas extsting tor 1g years, the clinical suspicion oflyli,glancV arising in benign tumor wai stLng.lhus the mass lesion was excised and sent forhistopathological examination.

Pathological examination: Gross examinationrevealed 10.5X8.5X7 cm well circumscribeOlobulated tumor. Cut surface ot tne tumoi wassolid, grayish white, glistening without

"*r. oinemorrhage and necrosis (Fig. 2a),

Microscopic examination revealed a wellcircumscribed tumor located in the O"rrnia.Tumor showed spindle cell proliferation- wiifrvarying c_ellularity and hem ang io_pericytoma likevessels. The hyper-cellular arels inow'eO spinJtecells arranged in fascicles whereas nypo_ci:ifuiirur?as

.. showed pattern-less patibrn wiin

-extracellular collagen deposition. fne celts hadeosinophilic cytoplasm, oval bland tooking nucLiwith minim.al mitoses (<Zj0 HpF). fne-r,ucieiipreomorphism was minimal and necrosis was

*l:l , . .(F,.s. pa, sb and 5"f.lmmunohistochemically tumor cells showe'dditfuse positivity lor CD34 and CD99 (Fig. 3d and3e), but were negative for 5100 iro-tei,

"nJdesmin, Based on histomorphology anOimmunohistochemistry the diagnosis oi'sotitarvtrbrous tumor was rendered.

3. DISCUSSION

SFT of soft tissues first described in 1931 bvKlemperer and Rabin is a rare entity If _101. Mo-Jt

ll1grs present as a stow growing faintesi mass[5], but some of the tumors maylause rocafiieJpain due to mass eftect [8].-fne OuratiJn oJextrathoracic solitary fibrous tumor ranges from 1Ioll!1o.6 years [1,2,8,9]. The tongeit durationfor SFT is 20 years which has Ueei r.eporteJlnparatesticular region by Lee et al. [1i].To ourKnowtedge present case study has longestduration of solitary fibrous tumor for .lg

Vru[ in

According to Wilky et al. [4], extra-thoracicsolitary fibrous tumors are more likely to havea_q/pical histologicalleatures. We repori SFT in a45 year male who had a tumor in neck forlongest duraticn of 1g years with borderlinehistomorphology

2. CASE PRESENTATION

A 4S-year-old non-smoker, non-alcoholic malepresented to surgical OPD with painless swellinoinvolving right side of neck. A small swelling wa!present in right side of neck since 18 iears;however there was rapid enlargemenf withlobulation over a period of one yeai. There wasno p.ast history of trauma, surgery or any majormedical illness. There was no history oi tevlr,dyspnea, night sweats or weight loss.

the neck region which has not been described inany case report till date.

SFT occurring in the soft tissue presents as awell circumscribed mass ranging iri size trom i-Ocm. Cut surface is usually solid, grey white topinkish brown with lack ot -neirosis

oihemorrhage ['1-3].

Microscopically this tumor has characteristicappearance of "patternless pattern" where thecells are haphazardly embedded in th;colagenous stroma [3-7]. lmmunohistochemi_cally . these cells show strong and diffusepositiirity for CD 34, CD g9 and viriabte po.itirityfor Bcl-2 [9,10]. The other patterns which can beobserved are short fascicles, storiform patt"rr,,

Agale et al.;BJMMR, 1t(p): t-6, ZOt6; Atticte 11.BJMMR.2O6AI

hemangio-pericytomatous pattern andtetomyoma- like pattern [S]. The vasculaturevaries from slit like and dilated vessels tobranching vessels with typical ,staonorn,appearance. The nuclei of spindle cells uluallyhave bland appearance with minimalpleomorphism and absent mitotic activity. Thisappearance of solitary fibrous tumor fras to bemainly difterentiated from the benign andmalignant neurogenic tumors, ,O"esmoiJ,

fibromatosis, and myofibroblastic sarcom". ifr.diagnoslic problems arise as this tumor is usuallynot considerecj in the differential diagnosiiclinically and pathologically and atso Oueiolnetact that morphologically it may mimic othertumors.

Fig. 1a. clinical photograph s.howing large lobulated mass in the neck withstretched out overlying skin

Fig' 1b and 1 c' cT scan of Head Neck region showed well detined lobulated lesion on the, right side of neck with enhancement in subcutan;;u"1L"r"

Fig.1b. Fig.1c.

Solitary neurofibroma presenls as a nodularlesion and histomorpholbgically is .nui""i"ri."OOy shredded carrot appearance with spindle cellsnavrng wavy buckled nuclei. lmmunohisto_chemically they are positive for 5_100

"ntiol"and are weakly positive for CD 84.

MPNST_morphologically can be differentiatedrro.m.sF I by presence of sweeping fascicles ofspindle cells with cellular' aid nu"f"",ple.omorphism and brisk mitotic activitv. irror.cells express S-100 antigen but are neiatire io,cD34 [6].

Low grade myolibroblastic sarcoma differs fromSFT by presence of diffuse infiftraiive jrowifrpattern with fascicles or storiform patteri. Thenuclei are fusiform with indentation anO smittnucleoli [12]. lmmunophenotypically tfrJ tumoicells are actin and desmin positive. Thev mavexpress CD34, CD99 focally which is in c6ntradtto diffuse expression seen in SFT 1121.

Desmoid type fibromatosis is distinctlvcharacterized by ill circumscribed mas,comprised of sweeping fascicles ot spindle ceilirn cotragenous stroma [1 3]. Like SFT thev havetow mitotic activity, prominent vasculature ind nonecrosis but immunohistochemically the cells areltlol-gtY positive for vimentin and aie negative foiCD34 and CD99.

Agale et at.;BJMMR, 11(2): 1_6,2016; Atticte no.BJMMR.20635

,t,:,:,1 ..ryqstasizins). catesory t6l. Sotitaryrorous tumor usually has a Oenign iorrse butmattgnant transformation is known lo occur. Thecriteria for malignancy jn SFT ,r"

"t Lrig" .i.,

{r'19. -qD_Ul High ceilutarity c) Miiotic "".tiritv(>4/1 0HPF) d) Necrosis [6].

ln the^studies by Vallat_Decouveleare et al. [g]and. Ganly et al. [5], lack of "ir"rrr.rloii,innuctear atypia, areas of increaseJ ;;lili;;it;'

necrosis and >4/10 HpF mitoses *"r" ,-*"rir'ias atypical features and predictive t".iJ,s ioimalignant behavior and high chancei oird$r;.ln. our case the tumor was large in size withl:b.yl1tio.l,_BO high ceilutarity, ro,i, mitot[ actiriiy(<4/10 HPF) and no necrosis. Also clinicatiirthere was sudden increase in tne size oi; I;#standing tumor, which indicates that this iumoimay.have high chances of recurrence;;dlh;;needs regular follow up.

A study by Witky et al. [4], supports that extra_rnoracic and ,,Borderline',

SFTs with anv ofEngland's criteria shoutd Oe conslUeieJ nilriris'r.tumors. As these SFTs have high recurren;" ;ri;and behave aggressively, sufueiltance- fi;;i.;Pl^_:llr-,.]o^years,. increasins rhe- r"i;;;;;;lnrervat atler 2 years is recommended [4].

According to recent ..yTg classification, Treatrirent for SFT is conservative surgery andextrapleural SFT is classified into intermediite resectability is tfre mo,-st important risk lactor.

Fig' 2' 2a and 2b' Grossly.tumor measured 10.5x8.5x7 cm with lobulated appearance. cutsurface was sorid greyish white gristeninjwith no ri".. ,i't ",i|ri[,rg" or necrosis

Agale et al.: BJMMR, fi(p): t-6,2016; Atlicte I}.BJMMR.2O6SI

Fig. 3aFig' 3a' Microscopic examinati.on showed spindre ceil tumor with ,,patternress pattern,,without necrosis (H&E, X10O)

Fig' 3b and 3c' Focally tumor showed high cellularity with spindle cells arranged in lascicles(H&E' X100);spindle_cells.had indistlncicytcptasmic margins and were embedded incollagenous matrix, nucrei were prump io ipiirdre with iil rit;fi; rctivity (H&E, X40)

Fig. 3d. CD 34 positivity ol tumor cells Fig. 3e. CD 99 positivity ol tumor cells

4. CONCLUSION

SFT is a rare tumor in neck region and has to bediflerentiated meticulously from other commonand rare soft tissue tumors. Close follow up isessential after complete surgical excision incases with "borderline" histomorphology.

CONSENT

All authors declare that written informed consentwas.obtained from the patient (or other approvedparties) for. publication of this case report anOaccompanying images.

ETHICAL APPROVAL

It is not applicable.

COMPETING INTEHESTS

Authors have declared that no competinginterests exist.

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