Primer Kutanöz Adenoid Kistik Karsinom
Özet
Primer kutanöz adenoid kistik karsinom (AKK), genellikle 60 yaş civarında ve sıklıkla kafa derisinde tek bir nodül olarak ortaya çıkan nadir ve malign bir deri eki tümörüdür. Histopatolojik olarak tükürük bezi AKK’una benzer şekilde kribriform, tübüler ve solid büyüme paternleri gösterir; miksoid veya hyalinize stroma içinde hiperkromatik çekirdekli abluminal hücreler ve perinöral invazyon eğilimi ile karakterizedir. İmmünohistokimyasal incelemelerde luminal hücrelerde EMA, CEA ve CAM 5.2 pozitifliği izlenirken, myoepitelyal diferansiyasyon gösteren hücrelerde S-100, aktin, kalponin, p63 ve SOX10 ekspresyonu saptanır; ayrıca olguların çoğunda CD117 ve MYB pozitifliği belirgindir. Ayırıcı tanıda bazal hücreli karsinomun adenoid kistik tipi, kribriform karsinom ve silindrom gibi tümörlerin dışlanması kritiktir. Olguların %60'ında hücre siklusu ve apoptozu etkileyen t(6;9) kromozom translokasyonu sonucu gelişen MYB-NFIB füzyon geni saptanır. Sistemik AKK'a kıyasla daha indolent bir seyir izleyen bu primer kutanöz neoplazm, uzak metastaz olasılığı düşük olsa da basit eksizyon sonrası %50'yi aşan yüksek bir lokal nüks oranına sahiptir; bu nedenle nüksleri önlemek adına mikroskopik kontrollü cerrahi (Mohs cerrahisi) tercih edilen etkin bir tedavi yöntemidir.
Primary cutaneous adenoid cystic carcinoma (ACC) is a rare malignant skin adnexal tumor that typically presents around the age of 60 as a solitary nodule, most commonly located on the scalp. Histopathologically resembling salivary gland ACC, it exhibits cribriform, tubular, and solid growth patterns, characterized by abluminal cells with hyperchromatic nuclei within a myxoid or hyalinized stroma, along with a distinct propensity for perineural invasion. Immunohistochemical analyses reveal EMA, CEA, and CAM 5.2 positivity in luminal cells, whereas S-100, actin, calponin, p63, and SOX10 expression confirm myoepithelial differentiation; furthermore, CD117 and MYB expression are prominently observed in the majority of cases. Differential diagnosis crucially requires distinguishing this tumor from the adenoid cystic variant of basal cell carcinoma, cribriform carcinoma, and cylindroma. Genetically, MYB gene activation through the recurrent t(6;9) translocation resulting in the MYB-NFIB fusion oncogene is identified in 60% of cases, altering cell cycle control and apoptosis. While primary cutaneous ACC follows a less aggressive course than systemic ACC with low metastasis rates, it carries a local recurrence rate exceeding 50% after standard excision; consequently, Mohs micrographic surgery represents the optimal treatment modality to prevent recurrences.
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