Papiller Ekrin Adenom
Özet
Papiller ekrin adenom (PEA), çoğunlukla distal ekstremitelerde soliter, yavaş büyüyen, kırmızı-kahverengi renkli bir dermal nodül olarak ortaya çıkan oldukça nadir ve benign bir ter bezi tümörüdür. İlk kez 1977 yılında tanımlanmış olan bu tümör, geniş bir yaş dağılımı sergilemekle birlikte sıklıkla kadınlarda ve ortalama 45 yaş civarında görülmektedir. Histopatolojik açıdan PEA, dermis tabakasında yerleşim gösteren, iyi sınırlı ancak kapsülsüz lezyonlardır; fibröz stroma içinde lümene doğru uzanan intraluminal mikropapiller projeksiyonlar barındıran ve çift sıralı küboidal hücrelerle döşeli dilate duktus benzeri yapılarla karakterizedir. İmmünohistokimyasal incelemelerde luminal hücrelerde CEA, EMA ve sitokeratinler pozitif boyanırken, bazal myoepitelyal katmanda S100, SMA, p63 ve kalponin pozitifliği saptanır. Ayrıca moleküler düzeyde vakaların yüzde 78'inde BRAFV600E mutasyonu tespit edilmiştir. Ayırıcı tanıda tübüler adenom, siringokistadenoma papilliferum ve özellikle malign bir antite olan agresif dijital papiller adenokarsinomun dışlanması kritik önem taşır. Tedavisinde temiz cerrahi sınırlarla komplet eksizyon veya Mohs mikrografik cerrahisi tercih edilir. Genel prognozu mükemmel olup metastaz riski bulunmamaktadır, ancak lezyonlar nadiren lokal rekürrens geliştirebilir.
Papillary eccrine adenoma (PEA) is a rare, benign sweat gland tumor that typically presents as a solitary, slow-growing, red-brown dermal nodule, predominantly located on the distal extremities. First described in 1977, this neoplasm exhibits a wide age distribution but is more frequently observed in females, with an average age of 45 years. Histopathologically, PEA presents as a well-circumscribed, unencapsulated dermal lesion characterized by dilated duct-like structures lined by a double layer of cuboidal epithelial cells, featuring intraluminal papillary projections of variable complexity within a fibrous stroma. Immunohistochemical evaluation demonstrates positivity for CEA, EMA, and cytokeratins in the luminal cells, whereas the basal myoepithelial layer expresses S100, SMA, p63, and calponin. At the molecular level, the BRAFV600E mutation is identified in 78% of cases. Differential diagnosis is essential to distinguish PEA from tubular adenoma, syringocystadenoma papilliferum, and particularly malignant counterparts like aggressive digital papillary adenocarcinoma. The standard treatment is complete surgical excision with clear margins, or Mohs micrographic surgery. The overall prognosis is excellent with no metastatic potential, although rare local recurrences may occur.
Referanslar
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