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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.\u

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