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<ul><li><p>65</p><p>CARCINOMUL MUCINOS CUTANAT PRIMAR- prezentare de caz -</p><p>MUCINOUS CARCINOMA PRIMARY CUTANEOUS- case report -</p><p>DELIA BOTEZATU*, DENISA URZICEANU*, MIHAELA OVARU*, IRINA TUDOSE*, MARIA GRIGORE**, SIMONA ROXANA GEORGESCU*</p><p>Summary</p><p>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. </p><p>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.</p><p>The clinical exam, histopathological examination andsystemic investigations confirmed the diagnosis of primarymucinous carcinoma of the skin. </p><p>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.</p><p>Key words: primary mucinous carcinoma of the skin,rare malignant tumor, cutaneous metastasis.</p><p>* Clinica de Dermato-Venerologie, Spitalul Clinic de Boli Infecioase i Boli Tropicale Prof. Dr. Victor Babe, BucuretiClinical Hospital of Infectious and Tropical Diseases Prof Dr. Victor Babe, Department of Dermato-Venereology, Bucharest</p><p>** Departamentul de histopatologie, Spitalul Clinic Prof. Dr. Victor Babe, BucuretiClinical Hospital Prof. Dr. Victor Babe, Department of Histopathology, Bucharest</p><p>Rezumat</p><p>Carcinomul mucinos cutanat primar este o tumormalign foarte rar ce afecteaz cel mai frecvent zonaperiorbital cu originea din zona profund a ductelorsudoripare ecrine. Acesta este dificil de difereniat deleziunile metastatice cutanate. </p><p>Prezentm cazul unui pacient de 78 de ani care seinterneaz pentru o leziune nodular asimptomatic,supraclavicular drept ce evolueaz de 3 ani; nu seevideniaz limfadenopatii n regiunea capului i gtului. </p><p>Pe baza examenului clinic, histopatologic i ainvestigaiilor sistemice s-a stabilit diagnosticul decarcinom mucinos cutanat primar. S-a practicat rezeciatumorii n limite de siguran oncologic. Pacientulrmne n evidena clinicii, fr semne de recidiv la 18 luni de la intervenie. Excizia n limite oncologicereprezint cea mai bun conduit terapeutic n cazulcarcinomului mucinos cutanat. </p><p>Cuvinte cheie: carcinom mucinos cutanat primar,tumor malign rar, metastaz cutanat.</p><p>CAZURI CLINICECLINICAL CASES</p><p>Intrat n redacie: 6.04.2015Acceptat: 5.05.2015</p><p>Received: 6.04.2015Accepted: 5.05.2015</p><p>Introducere</p><p>Carcinomul mucinos cutanat primar (CMCP)este o tumor malign extrem de rar ce derivdin zona profund a ductelor sudoripare ecrine[1]. Au fost citate mai puin de 200 de cazuri nliteratur, [2,3]. Tumora a fost pentru prima dat </p><p>Introduction</p><p>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 </p></li><li><p>66</p><p>DermatoVenerol. (Buc.), 60: 65-71</p><p>raportat de Lotzbeck n 1859, apoi descris dectre Lennox et al n 1952, iar mai trziuaprofundat de Mendoza i Helwig n 1971[4].</p><p>Caz clinic</p><p>Prezentm cazul pacientului R.D, n vrst de78 de ani, din mediul urban, Fitzpatrick tip II cese interneaz n clinica noastr pentru apariiaunui nodul rou-violaceu, asimptomatic, situat lanivelul fosei supraclaviculare drepte, cu oevoluie lent de circa 3 ani.</p><p>Din antecedentele personale patologicereinem HTA primar, stadiul II, sub tratament idiabet zaharat tip II n tratament cu antidiabeticeorale, antecedentele heredo-colaterale fiindnesemnificative. </p><p>Examenul obiectiv identific un pacientastenic, normoponderal, cu o TA medie de130/70 mmHg, compensat hemodinamic irespirator. </p><p>Examenul local relev formaiunea tumoral,nodular, solitar, bine-delimitat, de dimensiuniaproximative 0,7x0,4cm, de culoare roie-violacee, neulcerat, asimptomatic, cutelangiectazii vizibile pe suprafa, situat lanivelul fosei supraclaviculare drepte. La palpare,nodul mobil i ferm, fr adenopatii regionale.</p><p>Pe baza datelor anamnestice, examenuluiclinic i local ne putem orienta ctre undiagnostic diferenial de etap, ce include:carcinomul bazo-celular nodular, chistul epi-</p><p>first described by Lotzbeck in 1859, than byLennox et al in 1952 and later designated byMendoza and Helwig in 1971[4].</p><p>Clinical Case</p><p>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.</p><p>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. </p><p>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.</p><p>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</p><p>Fig. 1 Formaiune tumoral, nodular, solitar, bine-delimitat, roie-violacee, neulcerat, asimptomaticFig. 1 One, well-circumscribed, reddish non-ulcerated,asymptomatic nodule on the supraclavicular area</p><p>Fig. 2 HEx40 Celule tumorale dispuse n travee, cordoaneintegrate n acumulri de mucus, separate de septurifibrovasculare.Fig. 2 HEx40 Dermal strands of uniform-appearingtumour cells embedded in pools of mucin, separated by thinfibrovascular septae</p></li><li><p>67</p><p>DermatoVenerol. (Buc.), 60: 65-71</p><p>dermoid, hidrochistomul apocrin, carcinomulsebaceu, carcinomul spino-celular, melanomulamelanotic, pilomatrixomul.</p><p>S-a practicat biopsie excizional cu examenhistopatologic ce a decelat tumora format dincelule predominant bazaloide, cu citoplasmaPAS+, n travee anastomozate, plaje, cordoane igrupuri de celule izolate, marcat pleomorfismnuclear i numeroase mitoze atipice intra-tumoral; arii de difereniere adenoid; invazie ndermul profund cu interesarea jonciunii dermo-hipodermice i a limitei profunde de rezecie;zone de necroz tumoral; marcat infiltratinflamator polimorf i marcat reacie desmo-plazic intra i peritumoral evideniat lacoloraia Albastru alcian prin prezena de lacurintinse de mucus, epidermal supraiacent cu ariide atrofie i tergere a reliefului dermuluipapilar; fr conexiuni ntre epiderm i proli-ferarea tumoral.</p><p>Nu s-au efectuat teste de imunohistochimie.Pe baza rezultatului histopatologic, s-a</p><p>realizat diagnosticul diferenial ntre carcinomulmucinos cutanat primar i metastaza cutanat aadenocarcinomului (AC) mucinos. Pacientul afost ndrumat ctre clinica de medicin internpentru a i se efectua investigaiile suplimentare.Investigaiile sistemice pentru a gsi o eventualtumor malign cu o alt localizare au fost:radiografia toracic, ecografie abdominal itiroidian, CT abdominal, endoscopie digestiv</p><p>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. </p><p>Fig. 3 Alcianx40 - tumor format din celule predominantbazaloide, cu citoplasma Alcian+; masele de mucus semenin pozitive cu albastru Alcian.Fig. 3 Alcianx40 - Mucinous masses stained positive withAlcian blue stains</p><p>Fig. 4 Alcianx100 Pleomorfism nuclear i numeroasemitoze atipice intratumoral prezente; marcat infiltratinflamator polimorf i marcat reacie desmoplazic intra iperitumoral evideniat la coloraia Albastru Alcian prinprezena de lacuri ntinse de mucus, epidermal supraiacentcu arii de atrofie i tergere 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</p><p>Fig. 5 PASx100 Prezena lacurilor ntinse de mucusPAS+. n unele pri, celulele tumorale sunt organizate ncuiburi 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</p></li><li><p>68</p><p>DermatoVenerol. (Buc.), 60: 65-71</p><p>superioar i inferioar, dou teste hemocultconsecutive negative, iar nivelul antigenuluispecific al prostatei (PSA) i antigenul carcino-embrionar (CEA) au fost n limite normale.</p><p>S-a stabilit diagnosticul final de carcinommucinos cutanat primar pe baza examenuluihistopatologic i a investigaiilor sistemiceefectuate.</p><p>S-a reintervenit n clinica de chirurgiepracticndu-se rezecia chirurgical a tumorii nlimite de siguran oncologic (pn la nivelulplanului muscular, cu margini de siguran de 1.5cm).</p><p>Pacientul rmne n evidena clinicii noastre,fr semne de recidiv la 18 luni de laintervenie, excizia n limite oncologice fiind ceamai bun conduit terapeutic, cu att mai multcu ct datele din literatur descriu o rat derecuren local de 30%.</p><p>Discuii</p><p>Carcinomul mucinos cutanat primar esteacceptat ca fiind o tumor ce deriv din glandelesudoripare, clasificat ca o tumor malign, cudifereniere apocrin si ecrin[2]. CMCP este maifrecvent ntlnit la sexul masculin i apare deobicei la vrste cuprinse ntre 50 i 70 de ani. Dinpunct de vedere anatomic, tumora se ntlnetecu predilecie la nivelul capului i gtului,pleoapa fiind localizarea preponderent aacesteia n 41% din cazuri[3]. Alte localizridescrise sunt scalpul(17%), faa(14%), axila(9%),torace/abdomen(7%), gt(2%), ureche(1%)[3,5].Mendoza i Helwig au demonstrat primadescriere contemporan a acestei tumori culocalizare la nivelul pleoapei. Mai trziu, Wrighti Font au publicat cel mai mare studiu efectuatincluznd 21 de cazuri de CMCP al pleoapei. </p><p>Datorit raritii acestei tumori, un diagnosticcert nu poate fi fcut pn nu au fost efectuateinvestigaii suplimentare ce au exclus tumoramalign primar cu o localizare visceral careproduce mucin i poate metastaza la nivelulcutanat, cum ar fi cea de la nivelul snului,tractului gastro-intestinal, plmni, rinichi, ovar,pancreas i prostat. Leziunile metastatice cuorigine la nivelul snului sau colonului suntpredispuse frecvent s mimeze carcinomulmucinos al pielii[4].</p><p>We couldnt do the immunohistochemicalanalysis.</p><p>Based on histopathological findings, adifferential diagnosis between primary mucinouscarcinoma of the skin and metastatic mucinousadenocarcinoma was offered.</p><p>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. </p><p>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. </p><p>To ensure complete tumor removal, thepatient underwent surgery (scar resection till themuscular layer- in an oncological safe manner) insurgery department. </p><p>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).</p><p>Discussions</p><p>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.</p><p>Due to its rarity, an affirmative diagnosiscant be made until further investigations haveruled out other primary malignancies of otherorgans that may produce mucin and metastasizeto the skin, specifically, breast, lung, gastro-</p></li><li><p>69</p><p>DermatoVenerol. (Buc.), 60: 65-71</p><p>Clinic, pacienii pot prezenta frecvent unnodul, chist, o papul sau formaiune ulceratmic, solitar, asimptomatic, cu o evoluie lungde la 2-3 luni la civa ani. Nu exist constatareclinic specific pentru acest tip de tumor,aspectul variind de la un pacient la altul.Impresia clinic iniial este cea a unui chist,carcinom bazo-celular, keratoacantom, naevussau hidrochistom apocrin i n anumitecircumstane diferenierea clinic include leziunivasculare ca cele din sarcomul Kaposi[5]. </p><p>CMCP rar metastazeaz, dar rata derecuren local este mare (pn la 30% dincazurile descrise n literatur). Metastazeleregionale apar la o rat cuprins ntre 5-15%, iarcele la distan ntre 2-7% la pacienii afectai.Pacienii descriu o evoluie lent a leziunii de maimuli ani, complet asimptomatic. Ocazional,tumorile foarte vechi sau cele agresive pot invadastructurile adiacente[6]. Ipoteza cursului lent,benign al acestei tumori este corelat cuproducia de mucin (ce se leag de gradul naltde difereniere celular a acesteia). Mai mult,prezena lacurilor ntinse de mucus pot servi ca obarier fizic n extinderea tumorii, fcndcompresie pe stroma tumoral, mpiedicndcreterea, inhibnd sinteza de ADN i sczndrata angiogenezei[8].</p><p>Dei prezentarea clinic a CMCP este non-specific, examinarea histopatologic estepatognomonic. Tumora este de obicei binedelimitat, cu insule mici sau tubuli ai celulelorepiteliale ce plutesc n mucin. Mucina e separatprin septuri fine de fibre de colagen i estepozitiv la coloraia acid periodic Schiff (PAS),mucicarmina, albastru alcian la un pH de 2,5 ifier coloidal. Mucina a fost caracterizat ca isialomucina, fiind i sialidazo-labil. Celulelesunt mici, bazaloide, vacuolizate cu citoplasmeozinofilic. Pleomorfismul celular i mitozelesunt rare[7].</p><p>Diagnosticul diferenial ntre metastazacutanat a AC colo-rectal/sn sau cu o altlocalizare sus-amintit i CMCP este dificil,examenul histopatologic fiind similar n ambelesituaii. Investigaii suplimentare sunt necesare nvederea excluderii metastazei cutanate [7,8]. </p><p>Testele de imunohistochimie pot facilitadiagnosticul diferenial. Celule CMCP rmnpozitive pentru citokeratina (C...</p></li></ul>