Turkderm-Turk Arch Dermatol Venereol. 2014; 48(2): 100-104 | DOI: 10.4274/turkderm.53367

Primary Cutaneous Carcinosarcoma: The first reported case with peripheral nerve sheath differentiation

Pelin Yýldýz¹, Zeynep Tosuner¹, Ethem Güneren², Cuyan Demirkesen^3
1Department Of Pathology, Bezmialem Vakýf University, Medical Faculty, Istanbul, Turkey
²Department Of Plastic, Reconstructive And Aesthetic Surgery, Bezmialem Vakýf University, Medical Faculty, Istanbul, Turkey
³Department Of Pathology, Istanbul University, Cerrahpaþa Medical Faculty, Istanbul, Turkey,

Primary cutaneous carcinosarcomas (CS) are extremely rare biphasic tumors mainly located on sun-exposed areas. Two hypotheses–multiclonal (convergence) and monoclonal (divergence)- have been suggested for the evolution of the tumor. According to multiclonal hypothesis two or more stem cells of epithelial and mesenchymal origin give rise to these tumors, while a single totipotential cell differentiatiate into epithelial and mesenchymal components, either synchronously or metachronously according to monoclonal hypothesis. Cutaneous CSs are subdivided into two distinct groups as epidermal and adnexal CSs, due to their epithelial content. We present an interesting cutaneous adnexal CS, showing peripheral nerve sheath differentiation and having the spiradenocarcinoma component derived from spiradenoma. As far as we know, it is the first case of the literature with this features.

Keywords: Adnexal, cutaneous, carcinosarcoma; malignant peripheral nerve differentiation

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Primer Kutanöz Karsinosarkom: Literatürde malign periferik sinir kýlýfý tümörü diferansiasyonu gösteren ilk olgu

Pelin Yýldýz¹, Zeynep Tosuner¹, Ethem Güneren², Cuyan Demirkesen^3
1Bezmialem Vakýf Üniversitesi, Patoloji Anabilim Dalý, Ýstanbul, Türkiye
²Bezmialem Vakýf Üniversitesi, Plastik, Rekonstrüktif Ve Estetik Cerrahi Anabilim Dalý, Ýstanbul, Türkiye
³Ýstanbul Üniversitesi, Cerrahpaþa Týp Fakültesi, Patoloji Anabilim Dalý, Ýstanbul, Türkiye

Primer kutanöz karsinosarkomlar (KS) genellikle güneþe maruz kalan bölgelerde görülen çok nadir bifazik tümörlerdir. Tümörün geliþimi ile ilgili multiklonal (konverjans) ve monoklonal (diverjans) olmak üzere iki hipotez öne sürülmüþtür. Multiklonal hipoteze göre tümörün epitelyal ve mezenkimal iki ya da daha fazla kök hücreden, monoklonal hipoteze göre ise tek totipotential hücreden hem epitelyal hem de mezenkimal komponentlere, senkron ya da metakron olarak diferansiye olduðu savunulmaktadýr. Kutanöz KS’lar içerdikleri epitelyal komponentlerine göre epidermal ve adneksiyal tip olarak iki gruba ayrýlmaktadýr.Olgumuz malign periferik sinir kýlýfý yönünde diferansiasyon gösteren, spiradenomdan kaynaklanan spiradenokarsinom komponentine sahip, ilginç bir adneksiyal tip primer kutanöz karsinosarkom olgusudur.

Anahtar Kelimeler: Adneksiyal, kutanöz, karsinosarkom, malign periferik sinir diferansiasyonu

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