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65 CARCINOMUL MUCINOS CUTANAT PRIMAR - prezentare de caz - MUCINOUS CARCINOMA PRIMARY CUTANEOUS - case report - DELIA BOTEZATU*, DENISA URZICEANU*, MIHAELA ÞOVARU*, IRINA TUDOSE*, MARIA GRIGORE**, SIMONA ROXANA GEORGESCU* Summary Primary mucinous carcinoma is a rare malignant tumor that most frequently occurs in the periorbital area. This tumor originates from the deepest portion of the eccrine sweat duct. It is difficult to differentiate this tumor histologically from metastatic lesions. We present a 78 - years - old man with 3 years history of slowly augmentation, asymptomatic reddish nodule on the right supraclavicular area and presented in our clinic. No lymphadenopathy was evident in the head and neck region. The clinical exam, histopathological examination and systemic investigations confirmed the diagnosis of primary mucinous carcinoma of the skin. To ensure complete tumor removal, the patient underwent surgery in an oncological safe manner. He remains disease-free 13 months after surgical excision, surgery procedure appears to be a rational and effective treatment for this type of cutaneous tumour. Key words: primary mucinous carcinoma of the skin, rare malignant tumor, cutaneous metastasis. * Clinica de Dermato-Venerologie, Spitalul Clinic de Boli Infecþioase ºi Boli Tropicale “Prof. Dr. Victor Babeº”, Bucureºti Clinical Hospital of Infectious and Tropical Diseases “Prof Dr. Victor Babeº”, Department of Dermato-Venereology, Bucharest ** Departamentul de histopatologie, Spitalul Clinic “Prof. Dr. Victor Babeº”, Bucureºti Clinical Hospital “Prof. Dr. Victor Babeº”, Department of Histopathology, Bucharest Rezumat Carcinomul mucinos cutanat primar este o tumorã malignã foarte rarã ce afecteazã cel mai frecvent zona periorbitalã cu originea din zona profundã a ductelor sudoripare ecrine. Acesta este dificil de diferenþiat de leziunile metastatice cutanate. Prezentãm cazul unui pacient de 78 de ani care se interneazã pentru o leziune nodularã asimptomaticã, supraclavicular drept ce evolueazã de 3 ani; nu se evidenþiazã limfadenopatii în regiunea capului ºi gâtului. Pe baza examenului clinic, histopatologic ºi a investigaþiilor sistemice s-a stabilit diagnosticul de carcinom mucinos cutanat primar. S-a practicat rezecþia tumorii în limite de siguranþã oncologicã. Pacientul rãmâne în evidenþa clinicii, fãrã semne de recidivã la 18 luni de la intervenþie. Excizia în limite oncologice reprezintã cea mai bunã conduitã terapeuticã în cazul carcinomului mucinos cutanat. Cuvinte cheie: carcinom mucinos cutanat primar, tumorã malignã rarã, metastazã cutanatã. CAZURI CLINICE CLINICAL CASES Intrat în redacþie: 6.04.2015 Acceptat: 5.05.2015 Received: 6.04.2015 Accepted: 5.05.2015 Introducere Carcinomul mucinos cutanat primar (CMCP) este o tumorã malignã extrem de rarã ce derivã din zona profundã a ductelor sudoripare ecrine [1]. Au fost citate mai puþin de 200 de cazuri în literaturã, [2,3]. Tumora a fost pentru prima datã Introduction Primary mucinous carcinoma of the skin (PMCS) is an extremely rare adnexal tumor that is thought to originate from eccrine sweat glands [1]. There have been sporadic cases, less than 200 reported in English literature[2,3]. The tumor was

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Page 1: CARCINOMUL MUCINOS CUTANAT PRIMAR - prezentare de caz ... · - prezentare de caz - ... Spitalul Clinic de Boli Infecþioase ºi Boli Tropicale “Prof. Dr. Victor ... aprofundatã

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CARCINOMUL MUCINOS CUTANAT PRIMAR- prezentare de caz -

MUCINOUS CARCINOMA PRIMARY CUTANEOUS- case report -

DELIA BOTEZATU*, DENISA URZICEANU*, MIHAELA ÞOVARU*, IRINA TUDOSE*, MARIA GRIGORE**, SIMONA ROXANA GEORGESCU*

Summary

Primary mucinous carcinoma is a rare malignanttumor that most frequently occurs in the periorbital area.This tumor originates from the deepest portion of theeccrine sweat duct. It is difficult to differentiate this tumorhistologically from metastatic lesions.

We present a 78 - years - old man with 3 years historyof slowly augmentation, asymptomatic reddish nodule onthe right supraclavicular area and presented in our clinic.No lymphadenopathy was evident in the head and neckregion.

The clinical exam, histopathological examination andsystemic investigations confirmed the diagnosis of primarymucinous carcinoma of the skin.

To ensure complete tumor removal, the patientunderwent surgery in an oncological safe manner. Heremains disease-free 13 months after surgical excision,surgery procedure appears to be a rational and effectivetreatment for this type of cutaneous tumour.

Key words: primary mucinous carcinoma of the skin,rare malignant tumor, cutaneous metastasis.

* Clinica de Dermato-Venerologie, Spitalul Clinic de Boli Infecþioase ºi Boli Tropicale “Prof. Dr. Victor Babeº”, BucureºtiClinical Hospital of Infectious and Tropical Diseases “Prof Dr. Victor Babeº”, Department of Dermato-Venereology, Bucharest

** Departamentul de histopatologie, Spitalul Clinic “Prof. Dr. Victor Babeº”, BucureºtiClinical Hospital “Prof. Dr. Victor Babeº”, Department of Histopathology, Bucharest

Rezumat

Carcinomul mucinos cutanat primar este o tumorãmalignã foarte rarã ce afecteazã cel mai frecvent zonaperiorbitalã cu originea din zona profundã a ductelorsudoripare ecrine. Acesta este dificil de diferenþiat deleziunile metastatice cutanate.

Prezentãm cazul unui pacient de 78 de ani care seinterneazã pentru o leziune nodularã asimptomaticã,supraclavicular drept ce evolueazã de 3 ani; nu seevidenþiazã limfadenopatii în regiunea capului ºi gâtului.

Pe baza examenului clinic, histopatologic ºi ainvestigaþiilor sistemice s-a stabilit diagnosticul decarcinom mucinos cutanat primar. S-a practicat rezecþiatumorii în limite de siguranþã oncologicã. Pacientulrãmâne în evidenþa clinicii, fãrã semne de recidivã la 18 luni de la intervenþie. Excizia în limite oncologicereprezintã cea mai bunã conduitã terapeuticã în cazulcarcinomului mucinos cutanat.

Cuvinte cheie: carcinom mucinos cutanat primar,tumorã malignã rarã, metastazã cutanatã.

CAZURI CLINICECLINICAL CASES

Intrat în redacþie: 6.04.2015Acceptat: 5.05.2015

Received: 6.04.2015Accepted: 5.05.2015

Introducere

Carcinomul mucinos cutanat primar (CMCP)este o tumorã malignã extrem de rarã ce derivãdin zona profundã a ductelor sudoripare ecrine[1]. Au fost citate mai puþin de 200 de cazuri înliteraturã, [2,3]. Tumora a fost pentru prima datã

Introduction

Primary mucinous carcinoma of the skin(PMCS) is an extremely rare adnexal tumor that isthought to originate from eccrine sweat glands[1]. There have been sporadic cases, less than 200reported in English literature[2,3]. The tumor was

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raportatã de Lotzbeck în 1859, apoi descrisã decãtre Lennox et al în 1952, iar mai târziuaprofundatã de Mendoza ºi Helwig în 1971[4].

Caz clinic

Prezentãm cazul pacientului R.D, în vârstã de78 de ani, din mediul urban, Fitzpatrick tip II cese interneazã în clinica noastrã pentru apariþiaunui nodul roºu-violaceu, asimptomatic, situat lanivelul fosei supraclaviculare drepte, cu oevoluþie lentã de circa 3 ani.

Din antecedentele personale patologicereþinem HTA primarã, stadiul II, sub tratament ºidiabet zaharat tip II în tratament cu antidiabeticeorale, antecedentele heredo-colaterale fiindnesemnificative.

Examenul obiectiv identificã un pacientastenic, normoponderal, cu o TA medie de130/70 mmHg, compensat hemodinamic ºirespirator.

Examenul local relevã formaþiunea tumoralã,nodularã, solitarã, bine-delimitatã, de dimensiuniaproximative 0,7x0,4cm, de culoare roºie-violacee, neulceratã, asimptomaticã, cutelangiectazii vizibile pe suprafaþã, situatã lanivelul fosei supraclaviculare drepte. La palpare,nodul mobil ºi ferm, fãrã adenopatii regionale.

Pe baza datelor anamnestice, examenuluiclinic ºi local ne putem orienta cãtre undiagnostic diferenþial de etapã, ce include:carcinomul bazo-celular nodular, chistul epi-

first described by Lotzbeck in 1859, than byLennox et al in 1952 and later designated byMendoza and Helwig in 1971[4].

Clinical Case

We present the case of a 78-year old malepatient, Fitzpatrick type II, from the urban area,with 3 years history of slowly augmentation,asymptomatic reddish nodule on the rightsupraclavicular area presented in our clinic.

Personal history showed arterial hyper-tension type II under treatment and diabetesmellitus type II treated with oral anti-diabeticmedication. Clinical examination revealed a palepatient with a mean arterial pressure 130/70mmHg. Local exam pointed out one nodulartumor, well-circumscribed, 0.7x0.4 dimensions,reddish, non-ulcerated, asymptomatic, withtelangiectases on the surface, situated in the rightsupraclavicular area.

Based on personal history, general and localexam a differential diagnosis that includedepidermoid cyst, nodular/cystic basal cellcarcinoma, hidrocystoma, sebaceous carcinoma,squamous cell carcinoma, melanoma, pilo-maticoma was made.

Histological examination of a biopsyspecimen revealed dermal strands of uniform-appearing tumour cells embedded in pools ofmucin, separated by thin fibrovascular septae.Mucinous masses stained positive with periodic

Fig. 1 – Formaþiune tumoralã, nodularã, solitarã, bine-delimitatã, roºie-violacee, neulceratã, asimptomaticãFig. 1 – One, well-circumscribed, reddish non-ulcerated,asymptomatic nodule on the supraclavicular area

Fig. 2 HEx40 – Celule tumorale dispuse în travee, cordoaneintegrate în acumulãri de mucus, separate de septurifibrovasculare.Fig. 2 – HEx40 Dermal strands of uniform-appearingtumour cells embedded in pools of mucin, separated by thinfibrovascular septae

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dermoid, hidrochistomul apocrin, carcinomulsebaceu, carcinomul spino-celular, melanomulamelanotic, pilomatrixomul.

S-a practicat biopsie excizionalã cu examenhistopatologic ce a decelat tumora formatã dincelule predominant bazaloide, cu citoplasmaPAS+, în travee anastomozate, plaje, cordoane ºigrupuri de celule izolate, marcat pleomorfismnuclear ºi numeroase mitoze atipice intra-tumoral; arii de diferenþiere adenoidã; invazie îndermul profund cu interesarea joncþiunii dermo-hipodermice ºi a limitei profunde de rezecþie;zone de necrozã tumoralã; marcat infiltratinflamator polimorf ºi marcatã reacþie desmo-plazicã intra ºi peritumoralã evidenþiatã lacoloraþia Albastru alcian prin prezenþa de lacuriîntinse de mucus, epidermal supraiacent cu ariide atrofie ºi ºtergere a reliefului dermuluipapilar; fãrã conexiuni între epiderm ºi proli-ferarea tumoralã.

Nu s-au efectuat teste de imunohistochimie.Pe baza rezultatului histopatologic, s-a

realizat diagnosticul diferenþial între carcinomulmucinos cutanat primar ºi metastaza cutanatã aadenocarcinomului (AC) mucinos. Pacientul afost îndrumat cãtre clinica de medicinã internãpentru a i se efectua investigaþiile suplimentare.Investigaþiile sistemice pentru a gãsi o eventualãtumorã malignã cu o altã localizare au fost:radiografia toracicã, ecografie abdominalã ºitiroidianã, CT abdominal, endoscopie digestivã

acid-Schiff and Alcian blue stains. In some partstumour cells were organized into small-sizedglands. The cells had centrally placed roundnuclei with a moderate amount of cytoplasma.The nuclei showed a marked degree ofpleomorphism, vesicular chromatin, smallnucleoli and multiple mitosis.

Fig. 3 – Alcianx40 - tumorã formatã din celule predominantbazaloide, cu citoplasma Alcian+; masele de mucus semenþin pozitive cu albastru Alcian.Fig. 3 – Alcianx40 - Mucinous masses stained positive withAlcian blue stains

Fig. 4 – Alcianx100 – Pleomorfism nuclear ºi numeroasemitoze atipice intratumoral prezente; marcat infiltratinflamator polimorf ºi marcatã reacþie desmoplazicã intra ºiperitumoralã evidenþiatã la coloraþia Albastru Alcian prinprezenþa de lacuri întinse de mucus, epidermal supraiacentcu arii de atrofie ºi ºtergere a reliefului dermului papilar.Fig. 4 – Alcianx100 – The cells had centrally placed roundnuclei with a moderate amount of cytoplasma. The nucleishowed a marked degree of pleomorphism, vesicularchromatin, small nucleoli and multiple mitosis

Fig. 5 – PASx100 – Prezenþa lacurilor întinse de mucusPAS+. În unele pãrþi, celulele tumorale sunt organizate încuiburi de glande de mici dimensiuni.Fig. 5 – PASx100 – Mucinous masses stained positive withperiodic acid-Schiff. In some parts tumour cells wereorganized into small-sized glands

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superioarã ºi inferioarã, douã teste hemocultconsecutive negative, iar nivelul antigenuluispecific al prostatei (PSA) ºi antigenul carcino-embrionar (CEA) au fost în limite normale.

S-a stabilit diagnosticul final de carcinommucinos cutanat primar pe baza examenuluihistopatologic ºi a investigaþiilor sistemiceefectuate.

S-a reintervenit în clinica de chirurgiepracticându-se rezecþia chirurgicalã a tumorii înlimite de siguranþã oncologicã (pânã la nivelulplanului muscular, cu margini de siguranþã de 1.5cm).

Pacientul rãmâne în evidenþa clinicii noastre,fãrã semne de recidivã la 18 luni de laintervenþie, excizia în limite oncologice fiind ceamai bunã conduitã terapeuticã, cu atât mai multcu cât datele din literaturã descriu o ratã derecurenþã localã de 30%.

Discuþii

Carcinomul mucinos cutanat primar esteacceptat ca fiind o tumorã ce derivã din glandelesudoripare, clasificat ca o tumorã malignã, cudiferenþiere apocrinã si ecrinã[2]. CMCP este maifrecvent întâlnit la sexul masculin ºi apare deobicei la vârste cuprinse între 50 ºi 70 de ani. Dinpunct de vedere anatomic, tumora se întâlneºtecu predilecþie la nivelul capului ºi gâtului,pleoapa fiind localizarea preponderentã aacesteia în 41% din cazuri[3]. Alte localizãridescrise sunt scalpul(17%), faþa(14%), axila(9%),torace/abdomen(7%), gât(2%), ureche(1%)[3,5].Mendoza ºi Helwig au demonstrat primadescriere contemporanã a acestei tumori culocalizare la nivelul pleoapei. Mai târziu, Wrightºi Font au publicat cel mai mare studiu efectuatincluzând 21 de cazuri de CMCP al pleoapei.

Datoritã raritãþii acestei tumori, un diagnosticcert nu poate fi fãcut pânã nu au fost efectuateinvestigaþii suplimentare ce au exclus tumoramalignã primarã cu o localizare visceralã careproduce mucinã ºi poate metastaza la nivelulcutanat, cum ar fi cea de la nivelul sânului,tractului gastro-intestinal, plãmâni, rinichi, ovar,pancreas ºi prostatã. Leziunile metastatice cuorigine la nivelul sânului sau colonului suntpredispuse frecvent sã mimeze carcinomulmucinos al pielii[4].

We couldn’t do the immunohistochemicalanalysis.

Based on histopathological findings, adifferential diagnosis between primary mucinouscarcinoma of the skin and metastatic mucinousadenocarcinoma was offered.

Systemic investigations to search for aprimary tumor else where were negative andincluded: chest X-ray, ultrasonography of thethyroid gland, abdomen, total body computertomograph and upper and lower gastrointestinalendoscopy. Serum levels of prostate-specificantigen and carcinoembryonic antigen werenormal. Two consecutive stool samples for faecaloccult blood were also negative.

Based on the clinical history of a slow-growing mass of long duration and negativesystemic work-up for a primary lesion elsewhere, a diagnosis of primary mucinouscarcinoma of the skin was made.

To ensure complete tumor removal, thepatient underwent surgery (scar resection till themuscular layer- in an oncological safe manner) insurgery department.

He remains disease-free 18 months aftersurgical excision, surgery procedure appears tobe a rational and effective treatment for this typeof cutaneous tumour because of the high localrecurrence rate (30% of cases).

Discussions

PMCS is accepted to be a tumor thatoriginates from the deepest portion of the sweatsglands, currently classified as a malignant onewith apocrine and eccrine differentiation [2].Primary mucinous carcinoma of the skin isslightly more common in men and occurs morefrequently between the ages 50 and 70 years.Anatomically, it arises in the head and neckregion, the eyelid being the most commonlyaffected (41% of cases). Additional locationsinclude the scalp (17%), face (14%), axilla (9%),chest/abdomen (7%), neck (2%), ear (1%)[3,5].Mendoza and Helwig provided the firstcontemporary report of mucinous carcinoma ofthe eyelid. Soon there after, Wright and Fontpublished the largest study to date of mucinouscarcinoma of the eyelid (21 cases), including 2previously reported cases.

Due to its rarity, an affirmative diagnosiscan’t be made until further investigations haveruled out other primary malignancies of otherorgans that may produce mucin and metastasizeto the skin, specifically, breast, lung, gastro-

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Clinic, pacienþii pot prezenta frecvent unnodul, chist, o papulã sau formaþiune ulceratãmicã, solitarã, asimptomaticã, cu o evoluþie lungãde la 2-3 luni la câþiva ani. Nu existã constatareclinicã specificã pentru acest tip de tumorã,aspectul variind de la un pacient la altul.Impresia clinicã iniþialã este cea a unui chist,carcinom bazo-celular, keratoacantom, naevussau hidrochistom apocrin ºi în anumitecircumstanþe diferenþierea clinicã include leziunivasculare ca cele din sarcomul Kaposi[5].

CMCP rar metastazeazã, dar rata derecurenþã localã este mare (pânã la 30% dincazurile descrise în literaturã). Metastazeleregionale apar la o ratã cuprinsã între 5-15%, iarcele la distanþã între 2-7% la pacienþii afectaþi.Pacienþii descriu o evoluþie lentã a leziunii de maimulþi ani, complet asimptomaticã. Ocazional,tumorile foarte vechi sau cele agresive pot invadastructurile adiacente[6]. Ipoteza cursului lent,benign al acestei tumori este corelatã cuproducþia de mucinã (ce se leagã de gradul înaltde diferenþiere celularã a acesteia). Mai mult,prezenþa lacurilor întinse de mucus pot servi ca obarierã fizicã în extinderea tumorii, fãcândcompresie pe stroma tumoralã, împiedicândcreºterea, inhibând sinteza de ADN ºi scãzândrata angiogenezei[8].

Deºi prezentarea clinicã a CMCP este non-specificã, examinarea histopatologicã estepatognomonicã. Tumora este de obicei binedelimitatã, cu insule mici sau tubuli ai celulelorepiteliale ce plutesc în mucinã. Mucina e separatãprin septuri fine de fibre de colagen ºi estepozitivã la coloraþia acid periodic Schiff (PAS),mucicarmina, albastru alcian la un pH de 2,5 ºifier coloidal. Mucina a fost caracterizatã ca ºisialomucina, fiind ºi sialidazo-labilã. Celulelesunt mici, bazaloide, vacuolizate cu citoplasmãeozinofilicã. Pleomorfismul celular ºi mitozelesunt rare[7].

Diagnosticul diferenþial între metastazacutanatã a AC colo-rectal/sân sau cu o altãlocalizare sus-amintitã ºi CMCP este dificil,examenul histopatologic fiind similar în ambelesituaþii. Investigaþii suplimentare sunt necesare învederea excluderii metastazei cutanate [7,8].

Testele de imunohistochimie pot facilitadiagnosticul diferenþial. Celule CMCP rãmânpozitive pentru citokeratina (CK) 7 ºi negative

intestinal tract, kidney, ovaries, pancreas andprostate. Metastatic lesions from the breast orcolon are most likely to mimic mucinouscarcinoma of the skin.

Clinically, patients usually present withsmall, solitary, asymptomatic papules, nodules,cysts or ulcerated lesions that have been presentfrom several months to years. There are nospecific clinical findings that are particularlycharacteristic of mucinous carcinoma; the clinicalappearance may vary from patient to patient.Initial clinical evaluation generally yields adifferential diagnosis that includes epidermoidcyst, cystic basal cell carcinoma, kerato-acanthoma, nevus or apocrine hidrocystoma and,in some instances, the clinical differentialincludes vascular lesions such as Kaposisarcoma[5].

Tumors rarely metastasize, but the localrecurrence rate is high (30% of cases). Regionalmetastasis occurs at a rate of 5 to 15 percent anddistant metastasis occurs in only 2 to 7% ofaffected patients. Patients have been describedwho have had a lesion for many years withoutseeking treatment and yet still rarely experiencesymptoms. Occasionally, longstanding neo-plasms or more aggressive tumors may invadeinto adjacent structures [6]. The generally benign,slow-growing course of mucinous carcinoma ishypothesized to be correlated with the pro-duction of mucin, which may correlate with a high degree of cellular differentiation.Additionally, the presence of copious amounts ofmucin may serve as a physical barrier to spread,compressing the tumor stroma and restrictinggrowth, as well as inhibiting DNA synthesis and,hence, decreasing the rate of angiogenesis [8].

Although the clinical presentation ofmucinous carcinoma is nonspecific, the histologicand pathologic examination is very characteristic.Tumors are generally well circumscribed withsmall islands or tubules of epithelial cells floatingin large pools of mucin. The mucin is separatedby thin fibrocollagenous septa and is positivelystained with periodic acid Schiff (PAS),mucicarmine, alcian blue at pH 2.5 and colloidaliron. The mucin has been characterized assialomucin; it is however sialidase-labile. Cellsare small, cuboidal and vacuolated witheosinophilic cytoplasm. Mitoses and cellularpleomorphism are rare. [7]

Differential diagnosis between PMCS andcutaneous metastasis particularly of gastro-intestinal and breast origin is difficult to made,further investigations are required.

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pentru CK 20, similar AC mucinos al sânului, dardiferite faþã de AC mucinos colo-rectal – CK7negative ºi CK20 pozitive. Astfel, absenþacitokinei(CK) 20 exclude metastaza cutanatã ceprovine de la AC mucinos colo-rectal. UtilizareaCK7 ºi CK20 prin coloraþii imunohistochimice nepermite diagnosticarea a aproximativ jumãtatedin carcinoamele mucinoase cu localizare visce-ralã, facilitând diagnosticul final. Alte tumoriCK7+ ºi CK20- ca AC de la nivelul plãmânuluisau vezicii biliare pot da de asemenea, metastazecutanate. Acestea pot fi excluse prin investigaþiisistemice suplimentare ºi utilizarea altor coloraþiiimunohistochimice specifice lor[9].

Utilizarea expresiei p63 prin imuno-histochimie în diagnosticul CMCP este contro-versatã deoarece ºi metastaze cutanate cu originemamarã ºi pulmonarã pot exprima aceastãproteinã, investigaþiile suplimentare adiþionalefiind obligatorii. Cazuri de CMCP au fost gãsite afi estrogen, progesteron si GCFDP-15 pozitive[8,9]. Quereshi et al sugereazã cã pozitivareatumorii cutanate pentru p63/CK5/6 ajutã înexcluderea metastazei cutanate cu originemamarã [8]. Într-o analizã complexã a meta-stazelor cutanate, Brownstein et al au descoperitcã doar 6% dintre acestea erau localizate lanivelul capului ºi gâtului[10].

Tratamentul CMCP impune excizie chirur-gicalã localã. Datoritã ratei de recidivã localãînaltã, excizia adecvatã cu margini de siguranþãoncologicã (cel puþin 1cm) este recomandatã. Aufost citate câteva cazuri tratate cu succes princhirurgie Mohs, un caz descris, tratat cu succesprin chirurgie Mohs ºi utilizarea markerilorimunohistochimici cu greutate molecularã micã.Alte tratamente ca radio ºi chimioterapia nu sunteficiente în cazul acestui tip de tumorã[7-10].Pacienþii sunt informaþi de importanþa contro-lului periodic în ceea ce priveºte recurenþa localãsau apariþia limfadenopatiilor regionale.

Concluzii

CMCP este o tumorã rarã de naturã malignãce trebuie evaluatã ºi tratatã corect. Stabilirea cucertitudine a diagnosticului final se face prinexamen histopatologic, investigaþii sistemicesuplimentare, +/- teste de imunohistochimie,urmate de un tratament chirurgical radical cu

Immunohistochemical analysis of PMCS mayhelp in differentiation of cutaneous metastases ofinternal neoplasm. The tumor cells of primarymucinous carcinoma stain positively for CK7 andnegative for CK20, similar to breast cancer, butdifferent from gastrointestinal adenocarcinomawhich is CK7 negative and CK20 positive. Use ofCK7 and CK20 immunostains thus allowsapproximately one-half of cases of mucinouscarcinomas to be effectively eliminated fromconsideration. Other CK7-positive and CK20-negative tumors such as adenocarcinoma of thelung and gallbladder may metastasize to the skin;these can be differentiated from a primary skinform based largely on clinical investigation withpotential assistance from other immuno-histochemical stains [9].

The expression of p63 by PMCS and itsdiagnostic utility are controversial due to itspresence also on cutaneous metastases of breastand lung adenocarcinoma, further systemicinvestigations being mandatory. Cases ofprimary mucinous carcinoma of the skin havebeen found to be estrogen receptor, progesteronereceptor and GCDFP-15 positive. Quereshi et alsuggest that finding an in-situ component oftumor that stains positive for p63 and CK5/6 canhelp to exclude metastatic mucinous breastcarcinoma [8]. Brownstein et al found that only6% were located on the head and neck region[10].

Treatment of mucinous carcinoma entailslocal excision. Because of the high rate ofrecurrence, adequate excision with generousmargins (at least 1cm) is recommended. Severalreports of successful treatment using Mohsmicrographic surgery have been described; onesuccessfully treated case used low-molecularweight immunostaining in the Mohs’s sections.Other treatments, such as chemotherapy andradiation, generally are not employed in themanagement of these tumors [7-10]. Patientsshould be counseled about the importance offrequent follow-up, for evaluation for local tumorrecurrence or development of regionallymphadenopathy.

Conclusions

PMCS is a rare malignant tumor which mustbe correct evaluated and treated. The practitionerestablishes an accurate diagnosis based on histo-pathological findings, additional systemic inves-tigations, +/- immunohistochemical analysis

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margini de siguranþã oncologicã sau chirurgieMohs ºi un follow-up periodic[8,9].

De subliniat în cazul pacientului nostru,progresia lentã a leziunii, examenul clinic localnespecific, localizarea rarã la nivelul foseisupraclaviculare ºi rezultatul histopatologic,toate fiind compatibile cu diagnosticul de CMCP.În plus, pacientul a fost complet investigatsistemic în clinica de medicinã internã în vedereadescoperirii unei malignitãþi viscerale, darrezultatele au fost negative. Rãmâne în evidenþaclinicii noastre, la 18 luni de la exciziachirurgicalã, fãrã semne de recidivã localã sauadenopatii regionale.

succeeded by radical surgical treatment andfrequent follow-up [8,9].

Particularly in our case is to remark theprogressive evolution of the lesion, the localclinical non-specific appearance, the supra-clavicular area as localization and the histologicfindings; all of these are consistent with thediagnosis of primary mucinous carcinoma of theskin. Further more, our patient underwentcomplete systemic evaluation for other internalmalignancies that may manifest as metastaticdisease to the skin, but the work-up wasnegative. He remains under our observation; norecurrence has been detected during follow-upperiod of 18 months.

Bibliografie/Bibliography

1. Weber PJ, Hevia O, Gretzula JC, Rabinovitz HC. Primary mucinous carcinoma, J Dermatolog Surg Oncol 1988,14:170-2.

2. Pilgrim JP, Kloss SG, Wolfish PS, Heng MC. Primary mucinous carcinoma of the skin with metastases to thelymph nodes. Am J Dermatopathol 1985; 7:461-9.

3. Urso C, Bondi R, Paglierani M, Salvadori A, Anichini C, Giannini A. Carcinomas of sweat glands, report of 60cases. Arch Pathol Lab Med 2001; 125:498-505.

4. Smith CC Metastazing carcinoma of the sweat-glands. Br J Surg 1955, 43 (177)80-84. 5. Breiting L, Christensen L, Dahlstrom K, Breiting V, Winther JF. Primary mucinous carcinoma of the skin: A

population based study. Int J Dermatol. 2008; 47:242-5. 6. Karimpour DJ, Johnson TM, Kang S, Wang TS, Lowe L. Mucinous carcinoma of the skin, J Am Acad Dermatol 1997;

36:323-6.7. Aijthkumar TV, Nileena N, Abraham EK, James FV, Nair MK. Bone marrow relapse in primary mucinous

carcinoma of the skin. Am J Clin Oncol 1999; 22:303-4.8. Kelly Brent C, Koay J, Driscoll MS, Raimer SS, Colome-Grimmer MI. Report of a case: primary mucinous

carcinoma of the skin, Dermatol On J, 14(6), 2008.9. Papalas JA, Proia AD. Primary mucinous carcinoma of the eyelid, a clinicopathologic and immunohistochemical

study of 4 cases and an update on recurrence rates; Arch Ophthalmol 2010; 128(9):1160-1165.10. Brownstein MH, Helwig EB. Metastatic tumours of the skin. Cancer. 1972; 29:1298-307.

Conflict de interese Conflict of interestNEDECLARATE NONE DECLARED

Adresa de corespondenþã: Delia BotezatuSpitalul Clinic “Prof. Dr. Victor Babeº”, Bucureºti – ªoseaua Mihai Bravu nr 281; e-mail: [email protected]

Correspondance address: Delia BotezatuClinical Hospital “Prof. Dr. Victor Babes”, Bucharest – Mihai Bravu street, no 281; e-mail: [email protected]