Tübüler Apokrin Adenom
Özet
Tübüler apokrin adenom (TAA), apokrin ter bezlerinden köken alan ve nadir görülen benign (iyi huylu) bir deri eki tümörüdür. İlk kez 1972 yılında tanımlanan bu neoplazi, Dünya Sağlık Örgütü (DSÖ) 2018 sınıflandırmasında apokrin tümörler başlığı altında yer almaktadır. Genellikle orta yaşlı kadınlarda ve en sık baş-boyun bölgesindeki saçlı deride ortaya çıkar. Klinik olarak dermis veya subkutan dokuda yerleşen, yavaş büyüyen, ağrısız ve iyi sınırlı soliter nodüller şeklinde izlenir. TAA sıklıkla organoid nevüs (nevus sebaseus) veya siringokistadenoma papilliferum gibi diğer hamartomatöz ve dermatolojik lezyonlarla birlikte gelişim gösterebilir. Makroskopik olarak sarı veya gri-beyaz renkli, kapsülsüz dermal kitleler olan bu tümörlerin histopatolojik incelemesinde lobüler bir mimari dikkat çeker. Lobüller, iç tabakasında apokrin farklılaşmanın patognomonik bir göstergesi olan dekapitasyon tipi sekresyon sergileyen silindirik hücrelerin, dış tabakasında ise basık miyoepitelyal hücrelerin bulunduğu çift sıralı tübüler yapılardan oluşur. İmmunohistokimyasal analizlerde luminal hücrelerde CK-7, EMA ve CEA pozitifliği saptanırken, dış miyoepitelyal tabaka p63 ve SMA ile gösterilir. Moleküler çalışmalarda vakaların bir kısmında BRAFV600E mutasyonu bildirilmiştir. Tedavisi, nüks riskini önlemek amacıyla geniş cerrahi sınırlarla yapılan eksizyondur ve prognozu mükemmeldir.
Tubular apocrine adenoma (TAA) is a rare, benign skin adnexal neoplasm originating from the apocrine sweat glands. First described in 1972, this tumor is classified under apocrine tumors in the 2018 World Health Organization (WHO) classification of skin tumors. It predominantly affects middle-aged women and is most commonly located on the scalp within the head and neck region. Clinically, TAA presents as a slow-growing, painless, well-circumscribed, solitary dermal or subcutaneous nodule. It frequently coexists with other dermatological lesions, such as organoid nevus (nevus sebaceus) or syringocystadenoma papilliferum. Macroscopically, it appears as a yellow or grayish-white, unencapsulated solid mass. Histopathologically, the tumor exhibits a distinct lobular architecture composed of well-differentiated tubular structures. These tubules are lined by a double layer of epithelial cells: an inner layer of columnar cells showing decapitation secretion, which is pathognomonic for apocrine differentiation, and an outer layer of flattened myoepithelial cells. Immunohistochemically, luminal cells express CK-7, EMA, and CEA, while the myoepithelial layer is highlighted by p63 and SMA. Molecular analyses have revealed BRAFV600E mutations in a significant subset of cases. Complete surgical excision with wide margins remains the definitive treatment, and the overall prognosis is excellent with rare recurrences.
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