Tumor in the plantar region: dermatofibrosarcoma protuberans in an infrequent topography,

AnBrasDermatol.2020;95(6):728---730

Anais

Brasileiros

de

Dermatologia

DERMATOPATHOLOGY

Tumor

in

the

plantar

region:

dermatofibrosarcoma

protuberans

in

an

infrequent

topography

夽,夽夽

Simone

Perazzoli

_,

_Renan

_Rangel

_Bonamigo

_,

_Renata

_Heck

_,

André

da

Silva

Cartell

a_{SanitaryDermatologyOutpatientClinic,PortoAlegre,RS,Brazil}

b_{FacultyofMedicine,UniversidadeFederaldoRioGrandedoSul,PortoAlegre,RS,Brazil}

c_{SanitaryDermatologyService,SecretariaEstadualdeSaúdedoEstadodoRioGrandedoSuleSantaCasadePortoAlegre,Porto}

Alegre,RS,Brazil

d_{MedicalResidenceinPathologicalAnatomy,HospitaldeClínicas,PortoAlegre,RS,Brazil}

Received27January2020;accepted2May2020

Availableonline16August2020

KEYWORDS Dermatofibrosar-coma; Immunohistochem-istry; Sarcoma

Abstract Dermatofibrosarcomaprotuberansisararemesenchymaltumor;itislocally

aggres-siveandpresentshighratesoflocalrecurrence.Itmaypresentasanodularorplaquevegetating

lesion.Itmainlyaffects thetrunk andproximallimbs,being rareinthe distalextremities.

Biopsyandimmunohistochemistryhelpconfirmthediagnosis.Theauthorsreportacaseof

der-matofibrosarcoma protuberanswith plantarregioninvolvement,ararepresentation.Tothe

bestoftheauthors’knowledge,only11casesofinvolvementofthefeetweredescribedinthe

internationalliterature.

©2020PublishedbyElsevierEspa˜na,S.L.U.onbehalfofSociedadeBrasileiradeDermatologia.

ThisisanopenaccessarticleundertheCCBYlicense(http://creativecommons.org/licenses/

by/4.0/).

夽 _{How to cite this article: Perazzoli S, Bonamigo RR, Heck}

R, Cartell AS. Tumor in the plantar region: dermatofibrosar-comaprotuberansinaninfrequenttopography.AnBrasDermatol. 2020;95:728---730.

夽夽_{Study conducted at the Sanitary Dermatology Outpatient}

Clinic,PortoAlegre,RS,Brazil.

∗_{Correspondingauthor.}

E-mail:simoneperazzoli@hotmail.com(S.Perazzoli).

Introduction

Dermatofibrosarcomaprotuberans(DFSP)isamesenchymal neoplasm of slowgrowth,locally aggressiveandwith low metastaticpotential.It hasseveralpresentationsand can becharacterizedasahardenedplaque,nodule,or vegetat-inglesion.1_{Localtraumahasbeendescribedasapotential}

risk factor for tumor onset.2 _{The most common sites of}

involvement are thetrunk (40%---50%), the proximal limbs (30%---40%), and the head and cervical region (10%---15%). In the literature review, 11 reports of tumors located in theplantarregionwereretrieved.Duetotherarityofthe

https://doi.org/10.1016/j.abd.2020.05.002

0365-0596/©2020PublishedbyElsevierEspa˜na,S.L.U.onbehalfofSociedadeBrasileiradeDermatologia.Thisisanopenaccessarticle undertheCCBYlicense(http://creativecommons.org/licenses/by/4.0/).

Tumorintheplantarregion 729 presentation onthedistal limbs,the authorsbelieve that

reportingthiscaseisrelevant.

Case

report

Malepatient,35yearsold,previouslyhealthy,complained of painful lesion on the left plantar region with progres-sivegrowthoversixmonths;hedeniedtrauma.Onphysical examination, he presented an erythematous, exophytic, vegetating, and ulcerated tumor on the first metatarsal joint(Fig.1). Histopathologicalexamination of thelesion revealedafusocellularproliferationwithafocalstoriform pattern(Fig.2). Intheimmunohistochemical study,a dif-fuse positivityfor CD34+cells wasobserved. The findings werecompatiblewithlow-gradefusocellularmesenchymal neoplasia,favoringthediagnosisofDFSP (Fig.3).Surgical treatmentinvolvedamputationofthehalluxandpartofthe leftforefoot;notumorrecurrencewasobservedafterfour monthsofpost-operativefollow-up.

Discussion

DFSPisararemesenchymaltumorwithlowratesof aggres-siveness. It presents slow progression, with high rates of localrecurrence andrarecases of distantmetastases.An Americanstudyconductedbetween2000and2010foundan incidenceof41casesin10millionpatients.3 _Somestudies

Figure1 Exophyticandulceratedtumorlesion.

Figure 2 Histopathology: fusocellular proliferation with a

focalstoriformpattern(Hemathoxylin&eosin×40)

Figure3 Immunohistochemistry:diffusepositivity forCD34

inspindlecellswithstoriformpattern.

observed a higher incidencein women andblack people. Itmostlyaffects thetrunk (40%---50%), theproximallimbs (30%---40%),andtheheadandcervicalregion(10%---15%).3

Aliteraturereviewpublishedin2019described11cases ofdermatofibrosarcoma thataffectedthefeet. Themean ageobservedwas41years;itwasmorefrequentinmenthan inwomen(8:3)andthemostfrequentlocationofthetumor wasthedorsumofthefeet.Themeangrowthperiodwas3.5 years.ThemeanDFSPsizeatdiagnosiswasapproximately 3.5cm.Clinicaldiagnosisisdifficultgiventhevarietyof pre-sentations.Dermoscopyhasbeendescribedasausefultool toaiddiagnosis.1_{Inareviewbasedontheanalysisof32}

der-matofibrosarcomas,themostcommonfeaturesdescribedin dermoscopywerethepresenceofvessels(81%),followedby apigmentnetwork(78%)andapinkishbackground(66%).3

Histologically,dermatofibrosarcoma presents monomor-phic spindle cells with little atypia and mitotic activity, arrangedinirregularandmultidirectional(storiform) fasci-cles.Thetumorinfiltratesthesubcutaneoustissue,creating the characteristic honeycomb pattern. The histological differential diagnoses of other spindle cell tumors are dermatofibroma, malignant fibrous histiocytoma, atypical fibroxanthoma,desmoplasticmelanoma,Kaposi’ssarcoma, andsolitaryfibroustumor.1

Immunohistochemistry becomes an important resource fordifferentialdiagnosis,asDFSPispositivefor CD34and negativeforS100protein,factorXIIIA,anddesmin.1_Allthe

casesdescribedintheliteratureweretestedforCD34and werepositive.3

The recommended treatment is lesion excision with 2---3cmofsafetymargins.4_{Involvementofmarginsis}

asso-ciatedwithlocalrecurrence.4_{Anothertherapeuticoptionis}

Mohsmicrographicsurgery.Managementwithradiotherapy andimatinibhasbeendescribed.Theuseofimatinibis indi-catedformetastases,localrecurrences,asneoadjuvancy,or whenthetumorisunresectable.5

Attentionshouldbepaidtothisdisease,eveninunusual anatomical areas, as the prognosis depends on the early diagnosisandtreatment.

Financial

support

730 PerazzoliSetal.

Authors’

contributions

Simone Perazzoli: Conception and planning of the study; elaborationand writingof the manuscript;criticalreview oftheliterature.

Renan Rangel Bonamigo: Approval of the final ver-sion of the manuscript; conception and planning of the study;elaborationandwritingofthemanuscript;obtaining, analyzing, and interpreting the data; effective participa-tion in research orientation; intellectual participation in propaedeuticand/ortherapeuticconductofstudiedcases; critical review of the literature; critical review of the manuscript.

Renata Heck: Approval of the final version of the manuscript;conception and planning of thestudy; elabo-rationandwritingofthemanuscript;obtaining,analyzing, and interpreting the data; intellectual participation in propaedeuticand/ortherapeuticconductofstudiedcases. André daSilvaCartell: Approvalof the finalversion of themanuscript;conceptionandplanningofthestudy; intel-lectual participation in propaedeutic and/or therapeutic conductofstudiedcases.

Conflicts

of

interest

Nonedeclared.

References

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2.Lee SW, Zaesim A, Jackson A, Borkat M. Fibrosarcomatous dermatofibrosarcomaprotuberans from scarfollowingtrauma. AutopsCaseRep.2018;8:e2018039.

3.MaddenC,SpectorA, SiddiquiS,MirkinG, YimJ,HaoX. Der-matofibrosarcomaprotuberansonadulttoes:acasereportand reviewoftheliterature.AnticancerRes.2019;39:2105---11.

4.GladdyRA,WunderJS.Risk-stratifiedsurveillancein dermatofi-brosarcomaprotuberans:lessismore.Cancer.2019;125:670---2.

5.Navarrete-DechentC,MoriS,BarkerCA,DicksonMA,NehalKS. Imatinibtreatmentforlocallyadvancedormetastatic dermatofi-brosarcomaprotuberans:asystematicreview.JAMADermatol. 2019;155:361---9.