Papillary intralymphatic angioendothelioma: Dabska tumor,

AnBrasDermatol.2020;95(2):214---216

Anais

Brasileiros

de

Dermatologia

DERMATOPATHOLOGY

Papillary

intralymphatic

angioendothelioma:

Dabska

tumor

夽,夽夽

Thadeu

Santos

Silva

_,

_Luciana

_Rebouc

_¸as

_de

_Araujo

_,

Geise

Rezende

Paiva

_,

_Rodrigo

_Guimarães

_Andrade

a_{DermatologyClinic,EscolaBahianadeMedicinaeSaúdePública,UniversidadeFederaldaBahia,Salvador,BA,Brazil} b_{PathologicalAnatomyService,Studart&StudartLaboratory,Salvador,BA,Brazil}

Received26August2018;accepted1March2019 Availableonline12February2020

KEYWORDS Histology; Lymphaticvessel tumors; Neoplasms; Softtissue neoplasms; Vasculartissue

Abstract Papillaryintralymphaticangioendothelioma(Dabskatumor)isararelymetastasizing lymphaticvascularneoplasmthatusuallyaffectschildrenandyoungadults.Themajorityof these cases occur insofttissues ofextremities,and todate less than40 cases havebeen described.Despitethegenerallyindolentevolution,canbelocallyinvasivewiththepotential tometastasize.Wedescribeacaseofayoungwomanpresentingwithaplantarlesion,for9 monthsandhistologicaldiagnosisofDabskatumor.Thisneoplasmshouldbeconsideredinthe differentialdiagnosisofvasculardermatoses,allowingearlydiagnosisandtreatment.Long-term follow-upshouldbeperformed.

©2020PublishedbyElsevierEspa˜na,S.L.U.onbehalfofSociedadeBrasileiradeDermatologia. ThisisanopenaccessarticleundertheCCBYlicense(http://creativecommons.org/licenses/ by/4.0/).

A35-year-oldfemalepatientsoughtadermatologist, com-plainingofaninjurytothesoleoftherightfoot9months prior.Shereportedfeelingonlyaslightsizeincrease.Upon clinicalexamination,therewasanareaofviolaceous stain-ing, with poorly defined limits, on the sole of the right foot (Fig. 1). Dermoscopy showed a homogeneous global pattern of violet color, with pigment distribution in

fur-夽 _{Howtocitethisarticle:SilvaTS,AraujoLR,PaivaGR,Andrade}

RG.Papillaryintralymphaticangioendothelioma:Dabskatumor.An

BrasDermatol.2020;95:214---6.

夽夽_{Studyconductedat theHospitalSantaIzabel, Salvador,BA,}

Brazil.

∗_{Correspondingauthor.}

E-mail:lureboucas.ufba@gmail.com(L.R.Araujo).

rowsandridges,aswellasdarkredspotsinsidethelesion (Fig.2).Anincisionalbiopsywasperformed,whose anato-mopathological study evidenced an intralymphatic lesion, withprominentpapillaryformationsandvascularaxeslined by rounded and hyperchromaticcells, projecting into the lumen (hobnailcells), compatiblewithpapillary intralym-phaticangioendothelioma---Dabskatumor (Figs.3and4). Widesurgicalexcisionwasindicatedandthepatient contin-uestobemonitored,withnosignofmetastasis.

Papillaryintralymphaticangioendothelioma(PILAor Dab-ska tumor) is a rare vascular neoplasm, defined as low grade malignancy tumor, with high tendency of local recurrence.1---3_{Originallyconsideredamalignanttumorand}

calledmalignantendovascularpapillary hemangioendothe-lioma, it was renamed in 1998, due to its borderline behavior,prominentpresenceoflymphaticvesselsandthe https://doi.org/10.1016/j.abd.2019.03.008

0365-0596/©2020PublishedbyElsevierEspa˜na,S.L.U.onbehalfofSociedadeBrasileiradeDermatologia.Thisisanopenaccessarticle

Papillaryintralymphaticangioendothelioma:Dabskatumor 215

Figure1 Violaceouslesion,withpoorlydefinedlimits,onthe soleoftherightfoot.

Figure2 Dermoscopyshowedhomogeneousglobalpatternof violaceousstainingwithblackenedreddots,distributedinside thelesion.

Figure3 Histopathology.Papillaryprojectionswithvascular axes lined by rounded and hyperchromatic cells --- ‘‘Hobnail cells’’(Hematoxylin&eosin,×100).

Figure4 Histopathology.Highermagnificationwithrounded andhyperchromaticcellsthatareprotrudingintothelumen ---‘‘hobnailcells’’(Hematoxylin&eosin,_×400).

presenceofintravascularpapillaryproliferation.2 _Todate,

less than 40 cases have been reported in the literature, mostlyaffecting soft tissue.2 _{Affects mainly children and}

youngadults,andmaybepresentatbirth,without predilec-tion for sex.2 _{Its diagnosis is often a challenge for the}

pathologistdue toits rarity,multifocalityand morpholog-icalcharacteristics.2_{Clinically,thelesionmaypresentasan}

intradermalnoduleordiscretesuperficialnodule,withslow growth,purplish,pinkorbluishcolorationandlarge varia-tioninsize(upto40cm).3_{Insomecases,itmaypresentwith}

superficialulcerationand/orsatellitenodules.2,3_Itismost

commonlylocatedinthedermisandsubcutaneouscellular tissueoftheextremities,andcanalsoaffectthetrunk,head andneck,withrare casesdescribedindeeper locations ---suchasspleen,tongue,testisandbones.2---6

Histopathologi-cally,thetumorischaracterizedbypresenting,inthedermis and/or subcutaneous tissue, thin-walled intercomposite vesselslinedbyendothelialhobnailcells,formingthe char-acteristicintraluminalpapillaryprojections,whichassume afocalpatterninrosettesor‘‘match-head’’.2,3,7

Glomeruli-like structures may be present.2,3,7 _{Mitoses are rare}

andnecrosisis absent.7,8 _{The immunohistochemical study}

demonstratespositivityforVEGFR-3andpodoplanin(D2-40) inthehobnailendothelialproliferations,indicativeof lym-phaticdifferentiation.8---10_{Thedifferentialdiagnosisismade}

withreactiveangioendotheliomatosis,benignintravascular endothelial hyperplasia and retiform hemangioendothe-lioma, which present negative immunohistochemistry for podoplanin(D2-40).3,9 _{Ithasagenerallyindolent}

develop-ment,however,itmaybelocallyinvasive,withrarereports of disseminationto regionallymph nodes and evendistal metastases.3 _{The gold standard treatment is wide}

surgi-calexcisionwithfreemargins,whichpresentsanexcellent prognosis.2,3,7 _{Thus, its recognition by the dermatologist}

becomesimportanttodetermineearlydiagnosisand treat-ment. Long-term clinical follow-up of these patients is mandatory.2,3,8---10

Financial

support

216 SilvaTSetal.

Authors’

contributions

Thadeu Santos Silva: Approval of the final version of the manuscript;conception andplanningofthestudy; obtain-ing, analysis, and interpretation of the data; intellectual participationinthe propaedeuticand/ortherapeutic con-ductofthestudiedcases;criticalreviewoftheliterature.

Luciana Rebouc¸as deAraujo:Approval ofthe final ver-sion of the manuscript; elaboration and writing of the manuscript;obtaining, analysis, andinterpretation of the data;criticalreviewoftheliterature;criticalreviewofthe manuscript.

GeiseRezendePaiva:Approvalofthefinalversionofthe manuscript;conceptionandplanningofthestudy;effective participationinresearchorientation;intellectual participa-tioninthepropaedeuticand/ortherapeuticconductofthe studiedcases;criticalreviewoftheliterature.

RodrigoGuimarãesAndrade:Approvalofthefinalversion of themanuscript; conception and planningof the study; obtaining,analysis,andinterpretationofthedata.

Conflicts

of

interest

Nonedeclared.

References

1.JoVY, DoyleLA. Refinementsinsarcomaclassification inthe current2013worldhealthorganizationclassificationoftumours ofsofttissueandbone.SurgOncolClinNAm.2016;25:621---43.

2.GambarottiM,RighiA,SbaragliaM,BianchiG,PicciP,Vanel D,etal. Intraosseouspapillaryintralymphatic angioendothe-lioma(PILA):onenewcaseandreviewoftheliterature.Clinical SarcomaRes.2018;8:1.

3.SchwartzRA,DabskiC,DabskaM.TheDabskatumor:a thirty-yearretrospect.Dermatology.2000;201:1---5.

4.Kugler A, Koelblinger P, Zelger B, Ahlgrimm-Siess V, Laimer M. Papillary intralymphatic angioendothelioma (PILA), also referredtoasDabskatumour,inan83-year-oldwoman.JEur AcadDermatolVenereol.2016;30:e59---61.

5.WardKA,EckerPM,WhiteRR,MelnikTE,GulbahceEH,Wilke MS,etal. Papillary intralymphaticangioendothelioma ofthe thigh:a case reportand reviewof the literature.Dermatol OnlineJ.2010;16:4.

6.Fanburg Smith JC, Michal M, PartanenTA, Alitalo K, Mietti-nenM. Papillary intralymphatic angioendothelioma (PILA): a reportof twelve cases of a distinctive vascular tumor with phenotypic features oflymphatic vessels.Am JSurg Pathol. 1999;23:1004---10.

7.DabskaM.Malignantendovascularpapillaryangioendothelioma ofthe skinin childhood. Clinicopathologicstudy of6 cases. Cancer.1969;24:503---10.

8.Bhatia A, Nada R, Kumar Y, Menon P. Dabska tumor (endovascular papillary angioendothelioma) of testis: a case reportwithbriefreviewof literature.DiagnPathol. 2006;1: 12.

9.Berni´c A, Novosel I,Krizanac S. Anunusual mole: an adult case of Dabska tumour. Coll. Antropol. 2012;36 Suppl. 2: 171---2.

10.NevesRI,Stevenson J,HanceyMJ,Vangelisti G, Miraliakbari R,MackayD,etal.Endovascularpapillaryangioendothelioma (Dabska tumor): underrecognized malignant tumor in child-hood.JPediatrSurg.2011;46:e25---8.