Trikoepitelyoma
Özet
Trikoepitelyoma (TE), kıl foliküllerinden köken alan, iyi huylu adneksiyal bir deri tümörüdür ve Dünya Sağlık Örgütü'nün 2018 sınıflamasında trikoblastoma başlığı altında toplanmıştır. Klinik olarak genellikle ergenlik sonrasında kadınlarda, yüz ve gövde üst kısmında cilt renginde papül veya nodüllerle ortaya çıkar; soliter, multipl ve desmoplastik alt tipleri mevcuttur. Multipl familyal formu otozomal dominant geçişli olup 16q12-q13 kromozomundaki CYLD gen mutasyonuyla ilişkilidir. Histopatolojisinde fibrotik stroma içinde uniform bazaloid hücre proliferasyonları, karakteristik horn kistleri ve papiller mezenkimal cisimcikler izlenir. İmmünhistokimyasal olarak CK5/6, CD10 ve CD34 pozitifliği, BerEP-4 ve androjen reseptör negatifliği ile karakterizedir. Özellikle morfolojik olarak çok benzediği bazal hücreli karsinomdan (BHK) ayrımında hücresel atipinin olmaması, spesifik foliküler stromanın varlığı ve CD10/CK20/BerEP-4 boyanma paternleri kritik rol oynar. Siringom ve mikrokistik adneksiyel karsinom da diğer önemli ayırıcı tanılarıdır. Malign dönüşümü oldukça nadir olan bu tümörün tedavisinde cerrahi eksizyon, elektrokoterizasyon, kriyoterapi ve lazer cerrahisi gibi yöntemler tercih edilmektedir.
Trichoepithelioma (TE) is a benign cutaneous adnexal tumor originating from hair follicles, categorized under trichoblastoma in the 2018 WHO classification. Clinically, it presents post-puberty, predominantly in females, as skin-colored papules or nodules on the face and upper trunk, with solitary, multiple, and desmoplastic subtypes. The multiple familial form exhibits autosomal dominant inheritance associated with CYLD gene mutations on chromosome 16q12-q13. Histopathologically, it features uniform basaloid cell proliferations within a fibrotic stroma, characteristic horn cysts, and papillary mesenchymal bodies. Immunohistochemically, TE shows positivity for CK5/6, CD10, and CD34, while being negative for BerEP-4 and androgen receptors. Distinguishing TE from basal cell carcinoma (BCC), which it closely resembles morphologically, relies on the absence of cellular atypia, the presence of a specific follicular stroma, and distinct CD10/CK20/BerEP-4 staining patterns. Syringoma and microcystic adnexal carcinoma are other critical differential diagnoses. Malignant transformation is exceedingly rare, and treatment options include surgical excision, electrocauterization, cryotherapy, and laser surgery.
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