TRICOBLASTOMUL NODULAR – O TUMORÃ RARÃ

NODULAR TRICHOBLASTOMA – A RARE TUMOR

IULIANA ELENA NIÞÃ*, CRISTINA MIHAELA CRISTEA***, ªTEFANA BÃETU*, MARIA MAGDALENA CONSTANTIN*,**

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Rezumat

Tricoblastomul este o tumorã benignã, rarã, de micidimensiuni, frecvent 1–2 cm, dezvoltatã din celulele germi-native ale foliculului pilos.

În Clinica de Dermatologie s-a prezentat un pacient învârstã de 55 de ani cu o formaþiune tumoralã nodularã lanivelul feþei, cu evoluþie lentã de aproximativ 1 an. Clinicºi dermatoscopic, ne-am orientat diagnosticul spre uncarcinom bazocelular, tricoepiteliom sau tricoblastom. S-aefectuat biopsia excizionalã a leziunii ºi diagnosticul a fostde tricoblastom cu hiperplazie reactivã de ducte ecrine.

Deoarece pânã în prezent nu existã suficiente studii înliteraturã care sã ajute la diagnosticul diferenþial dermato-scopic al tricoblastomului de carcinomul bazocelular sautricoepiteliom, gold-standardul rãmâne examinarea histo-patologicã.

Deºi excizia chirurgicalã este de prima intenþie întratarea tricoblastoamelor, în literaturã au fost raportate ºialte opþiuni, fãrã a fi susþinute de studii de siguranþã ºieficacitate, precum: electrodesicarea, terapia fotodinamicã,laserterapia CO2.

Tricoblastomul, deºi are o incidenþã redusã, nu trebuiepierdut din vedere atunci când facem diagnosticuldiferenþial al carcinomului bazocelular deoarece deciziaterapeuticã ºi prognosticul sunt diferite pentru cele douãtipuri de tumori.

Cuvinte cheie: tricoblastom, epiteliom bazocelular,tumori benigne.

* Clinica Dermatologie II, Spitalul Clinic Colentina, Bucureºti.2nd Department of Dermatology, Colentina Clinical Hospital, Bucharest.

** Universitatea de Medicinã ºi Farmacie „Carol Davila”, Bucureºti.„Carol Davila” University of Medicine and Pharmacy, Bucharest.

*** Universitatea Petrol-Gaze, Ploieºti.Petroleum-Gas University, Ploieºti.

CAZURI CLINICECLINICAL CASES

Summary

Trichoblastoma is a benign, rare, small-sized tumor,more commonly having 1-2 cm diameter, developed fromgerm cells of the hair follicle.

A 55-year-old male addressed to the DermatologyClinic for a nodular tumor on the face, with slow evolutionof about 1 year. Clinical examination and dermoscopy,imposed the following differential diagnosis: basal cellcarcinoma, trichoepithelioma or trichoblastoma. It wasperformed excisional biopsy and the diagnosis wastrichoblastoma with reactive hyperplasia of eccrine ducts.

Since there are not enough studies in the literatureabout dermoscopy of these tumors to make a cleardifferential diagnose, the gold standard remainshistopathological examination.

Although surgical excision is the first line treatmentfor trichoblastoma, other options have been reported in theliterature without being supported by safety and efficacystudies such as electrodessication, photodynamic therapy,CO2 laser.

Trichoblastoma, although has a reduced incidence,should be take into consideration when we make adifferential diagnosis of basal cell carcinoma because thetherapeutic decision and prognosis are different for the twotypes of tumors.

Key words: Trichoblastoma, basal cell epithelioma,benign tumors.

Intrat în redacþie: 28.04.2017Acceptat: 8.05.2017

Received: 28.04.2017 Accepted: 8.05.2017

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Introducere

Tricoblastomul este o tumorã benignã, rarã,de mici dimensiuni care se dezvoltã din celulelegerminative ale foliculului pilos, cu localizarefrecvent la nivelul extremitãþii cefalice. Forma-þiunea apare ca o papulã sau un nodul deculoarea pielii, care creºte lent.

Raportare de caz

Raportãm cazul unui pacient în vârstã de 55de ani care s-a prezentat pentru apariþia uneiformaþiuni tumorale nodulare, eritematoase,rotunde, bine delimitate, cu diametrul de 2 cm ºilocalizare la nivelul obrazului drept, având oevoluþie de aproximativ 1 an. Pacientul nu a pre-zentat alte leziuni cutanate ºi nu avea limfa-denopatii palpabile. Clinic ºi dermatoscopic, s-auavut în vedere diagnosticele de carcinom bazo-celular nodular, tricoepiteliom ºi tricoblastom. Înclinicã s-a efectuat biopsia excizionalã a leziunii ºidiagnosticul histopatologic a fost de trico-blastom cu hiperplazie reactivã de ducte ecrine.

Examenul histopatologic a descris forma-þiunea tumoralã nodularã, relativ bine delimitatã,cu aspect histopatologic de proliferare de celuleepiteliale bazaloide dispuse la nivelul dermuluiîn cordoane anastomozate sau muguri emergenþiaparent din structurile ductale ecrine; celuleleepiteliale relativ monomorfe, cu vagã palisadarea nucleilor în periferie, în centrul mugurilorepiteliali celule cu citoplasmã clarã; proliferareaepitelialã este distribuitã într-o stromã fibro-hialinizatã bogatã; marcat infiltrat limfocitarstromal, cu importantã extensie intraepitelialã lanivelul mugurilor epiteliali; ducte ecrine cutraiect sinuos intratumoral, unele cu siringo-metaplazie scuamoasã; epidermul peritumoralcu marcatã spongiozã ºi exocitozã limfocitarã,ostiumuri foliculare dilatate prin prezenþa dedopuri keratozice ºi colonii microbiene, adiacentmic chist epidermal. Aspectul histopatologic afost compatibil cu diagnosticul de tricoblastomcu hiperplazie reactivã de ducte ecrine.

Discuþii

Tricoblastomul este o tumorã rarã cutanatãcare se diferenþiazã cãtre germenul de pãr, pre-cursorul embrionar al foliculului pilos. Carci-

Introduction

Trichoblastoma is a rare, small, benign tumorthat develops from germ cells of the hair follicle,more frequent on cephalic extremity. It appears asa papule or a skin-colored nodule that growsslowly.

Case Report

We report the case of a 55-year-old patientwho presented a nodular tumor with 2-cmdiameter, erythematous, well-defined on theright cheek, with approximately 1 year evolution.The patient had no other skin lesions, norpalpable lymphadenopathy. Clinical examinationand dermoscopy suggested three differentialdiagnosis: nodular basal cell carcinoma, tricho-epithelioma and trichoblastoma. Excisionalbiopsy was performed and the histopathologicaldiagnosis revealed: trichoblastoma with reactivehyperplasia of eccrine ducts.

The histopathological showed a relativelywell-defined nodular tumor with proliferation ofbasal epithelial cells in the dermis grouped inanastomosis cords or emerging buds, apparentlyfrom eccrine ducts structures; relatively mono-morphic epithelial cells with vague palisade ofnuclei in the periphery, in the center of epithelialbuds, cells with clear cytoplasm; epithelialproliferation is distributed in a rich fibro-hyalinated stroma; Abundant stromal lympho-cytic infiltrate, with intraepithelial extension atthe level of epithelial buds; Eccrine ducts havinga sinuous tract located intratumoral, some ofthem with squamous syringometaplasia. Peri-tumoral epidermis have abundant spongiosisand lymphocytic exocytosis, dilated follicularostia in the presence of keratotic plugs andmicrobial colonies, and a small epidermal cyst.Histopathology was consistent with thediagnosis of trichoblastoma with reactivehyperplasia of eccrine ducts

Discussions

Trichoblastoma is a rare skin tumor thatdifferentiates itself from hair germ, theembryonic precursor of the hair follicle. Basal cell

nomul bazocelular, trichoblastomul ºi trico-epite-liomul – sunt derivate din celule germinativefoliculare. Deoarece are un comportamentbenign, diagnosticul diferenþial formulat peargumente clinice faþã de un carcinom bazo-celular ar putea evita exciziile inutile [1].

Tricoblastomul apare, în general, ca un nodulsolitar, de culoarea pielii, neulcerat, de 1–2 cmdiametru, cu telangiectazi superficiale [2].Aceastã tumorã a foliculilor piloºi rudimentaripoate lua diferite aspecte clinice ºi histo-patologice. Clinic poate apãrea ca o papulã deculoarea pielii sau ca un nodul. Patologic, poate finodular mic, nodular mare, retiform, racemiform,

carcinoma (BCC), trichoblastoma and tricho-epithelioma – are derived from follicular germcells. Because trichoblastoma is a benign tumor, agood clinical differential diagnosis between itand tBCC could avoid unnecessary excision [1].

Trichoblastoma generally appears as asolitary, skin-colored nodule, with 1–2 centi-meters diameter and superficial telangiectasia [2].This tumor of rudimentary hair follicles mayhave different clinical and histopathologicalappearances. On clinical examination, it mayappear as a skin-colored papule or as a nodule.Pathologically, it may be a small nodule, largenodule, retiform, racemiform, cribriform, or it

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Fig. 1. Tricoblastomul – aspect clinicFig. 1. Tricoblastoma - clinical aspect

Fig. 3. Derivarea proliferãrii constã din insule de celule bazaloidecu palisada perifericã în cadrul unui stromar fibrocelular similar

celui din jurul foliculilor. Numeroase limfocite din insuleletumorale ºi stroma. HE x 200

Fig. 3. Dermal proliferation consisting of islands ofbasaloid cells with peripheral palisading within a

fibrocellular stroma similar to that surrounding follicles.Numerous lymphocytes within the tumor islands and

stroma. HE x 200

Fig. 2. Nodul solitar, de culoarea pielii, neulcerat, de 1,2 /0.5 cm, cu telangiectazii superficiale arborizate pe un fond

perlatFig. 2. Unique nodule of 1.2 / 0.5 cm, skin-colored with

superficial telangiectasia on a pearly background

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cribriform sau poate lua formã de coloanã.Indiferent de tip, examenul histopatologic evidenþiazã celulele germinative foliculare(bazaloide) [3, 4].

Dermatoscopic, tricoblastomul poate pre-zenta telangiectazii arborizate pe fond alb perlat[2]. Chiar dacã telangiectaziile arborizate suntfrecvente în multe neoplasme cutanate, în cazulcarcinoamelor bazocelulare nodulare, telan-giectaziile sunt mai mari în calibru ºi au tendinþade a se ramifica mai mult. Spre deosebire decarcinomul bazocelular, tricoblastoamele (tricho-blastom, trichoepiteliom clasic ºi trichoepiteliomdesmoplastic) prezintã telagiectazii subþiri ºirareori ramificate [2, 5. 6].

Existã studii care afirmã cã cea mai izbitoarediferenþã dermatoscopicã dintre trichoblastom ºicarcinomul bazocelular tricoblastic (BCCt) estereprezentatã de cuiburile ovoide albastru-cenuºiiºi globulele gri-cenuºii, care sunt mai frecvente,dar nu prezente exclusiv în BCCt. Însã, datoritãasemãnãrii clinice ºi dermatoscopice dintre BCCtºi tricoblastom, histologia rãmâne elementul celmai important de diagnostic [1].

Tricoblastoamele prezintã telangiectazii arbo-rizate mici ºi fine, vase în formã de coroanã, fondperlat, chisturi milia, striuri albe, globuli maro,uneori ulceraþie. În carcinomul bazocelular,telangiectaziile arborizate sunt fine, scurte ºisuperficiale alãturi de ulceraþii sau eroziuni,structuri concentrice, structuri în formã de frunzãde arþar, spiþã de roatã, zone albe strãlucitoare,zone strãlucitoare roºu-lãptoase, linii ºi striurialbe. Carcinoamele bazocelulare pigmentare pre-zintã cuiburi ovale albastre-gri, globuli sau punctealbastre-gri sau maro-negre, val albastru-alb[2].

Deoarece pânã în prezent nu existã suficientestudii pentru a diferenþia dermatoscopic trico-blastomul de un carcinom bazocelular sau trico-epiteliom, gold-standardul rãmâne examinareahistopatologicã.

Histopatologic, sunt considerate variante detricoblastom: tricoepiteliomul, tricoepiteliomuldesmoplastic, limfadenomul cutanat (trico-blastom adamantinoid). Examenul histologicevidenþiazã în tricoblastom – papile foliculare ºibulbi, stromã abundentã cu fibrocite densgrupate. [2, 7, 8].

Histopatologic, tricoblastomul se diferenþiazãde tricoepiteliom prin localizarea sa în dermul

may take the form of a column. Regardless of thetype, the histopathological examination revealsfollicular germ cells (basal cells) [3].

On dermoscopy, trichoblastoma may presentarborising telangiectasia on a white pearl back-ground [2]. Although arborising telangiectasiaare common in many skin tumors, in nodularbasal cell carcinoma, telangiectasia are larger andtends to have more branches. Unlike BCC,trichoblastomas (trichoblastoma, classical tricho-epithelioma and desmoplastic trichoepithelioma)have thin and rarely branched telangiectasia [2, 5, 6].

There are studies stating that most visibledifferences on dermoscopy between tricho-blastoma and trichoblastic basal cell carcinomaare the existence of blue-gray ovoid nests the andblue-grey globules, which are more common butnot exclusively in tBCC. However, due to theclinial and dermatoscopic similarities betweentBCC and trichoblastoma, histology remains thegold standard for the diagnostic [1]. Tricho-blastoma have small and fine telangiectasia,crown-vessels, pearly appearance, white striae,brown globules, milia cysts, sometimes ulce-ration. In basal cell carcinoma are found: fine,short and superficial arborizing telangiectasia,ulceration or erosions, concentric structures,maple-leaf structures, spoke-wheel, milky-redareas, striae and white lines [2]. Still, currentlythere are insufficient studies to differentiate ondermoscopy trichoblastoma from basal cellcarcinoma or tricoepithelioma, as a result,histopathological examination remains the bestdiagnostic option. On histology, there arevariants of trichoblastoma: tricoepithelioma,desmoplastic tricoepithelioma, cutaneouslymphadenoma (adamantinoid trichoblastoma).Usually, histological examination shows intrichoblastoma - follicular papillae and bulbs,abundant stroma with densely clustered fibro-cytes. [7] [8] [2] On histopathological exam,trichoblastoma differentiates from tricho-epithelium by being localized in the deep dermis,while trichoepithelioma is located in thesuperficial dermis. [9] Unlike tBCC, tricho-blastoma is well defined by abuindantperitumoral stromal cells. Cells form trabeculae

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profund, în timp ce tricoepiteliomul este localizatîn dermul superficial [9].

Spre deosebire de BCCt, tricoblastomul estebine delimitat de celule stromale peritumoralemarcate. Celulele neoplazice formeazã cordoanecelulare neregulate sau trabecule ºi sunt aranjateîn palisade tipice la periferie. În tricoblastomlipseºte infiltratul inflamator, mitozele saunecroza (foarte rar pot apãrea mitoze saunecrozã). În carcinomul bazocelular tricoblasticmezenchimul care înconjoarã tumora este maipuþin abundent ºi structurat. Separarea tumoriiBCCt de stroma înconjurãtoare prin goluri ceapar goale optic, de obicei considerate artefactece apar în timpul procesãrii þesutului. BCCtprezintã infiltrat limfocitic, posibil ºi mitoze saunecrozã [1].

Tricoblastomul poate apãrea împreunã cunevi sebacei preexistenþi sau poate coexista cu unbazaliom [10]. Datoritã asocierii cu acesta dinurmã ºi a posibilitãþii de a se transforma în carci-nom tricoblastic, excizia chirurgicalã completãeste recomandatã. Alte variante terapeuticeraportate în literaturã, dar fãrã a fi susþinute destudii de siguranþã ºi eficacitate sunt: terapiafotodinamicã, terapia cu laser CO2, electro-desicarea. Momentan, terapia de elecþie pentruaceastã tumorã benignã, care aduce ºi beneficiireale de ordin estetic este excizia chirurgicalã.Recurenþe post excizie chirurgicalã au fost rarraportate.

Concluzii

Tricoblastomul, deºi are o incidenþã redusã,nu trebuie pierdut din vedere atunci când facemdiagnosticul diferenþial al carcinomului bazo-celular deoarece decizia terapeuticã ºi pro-gnosticul sunt diferite pentru cele douã tipuri detumori.

or irregular cell cords, neoplastic cells arearranged in palisades at the periphery. Tricho-blastoma lacks inflammatory infiltration, mitosisor necrosis (or they have very rare mitosis ornecrosis). In the trichoblastic basal cell carcinoma,the mesenchyma that surrounds the tumor is lessabundant or structured. BCCt shows lympho-cytic infiltration, and mitosis or necrosis arepossible. [1] Trichoblastoma may occur onsebaceous nevi or it may coexist with a basal cellcarcinoma. [10] Because trichobalstoma mayassociate with BCC, and because it maytransform into trickoblastic carcinoma, completesurgical excision is recommended. Othertherapeutic alternatives reported in the literature,but without sufficient safety and efficacy studiesare: photodynamic therapy, CO2 laser therapy,electrodessication. Currently, the therapy ofchoice for this benign tumor is surgical excision.Surgical recurrences have rarely been reported.

Conclusion

Trichoblastoma, although having a reducedincidence, should alsio be taken into consi-deration when we make differential diagnosis ofbasal cell carcinoma because the therapeuticdecision and prognosis are different for the twotypes of tumors.

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Conflict de interese Conflict of interestNEDECLARATE NONE DECLARED

Adresa de corespondenþã: Maria Magdalena ConstantinClinica de Dermatologie II, Spitalul Colentina BucureºtiE-mail: drmagdadinu@yahoo.com

Correspondance address: Maria Magdalena ConstantinDermatology II, Department, Colentina Clinical HospitalE-mail: drmagdadinu@yahoo.com

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