Siringokistadenoma Papilliferum
Özet
Siringokistadenoma papilliferum (SKAP), apokrin veya apo-ekrin bez kökenli olduğu düşünülen ve nadir izlenen benign bir deri eki tümörüdür. Olguların büyük kısmı doğumda ya da puberte öncesinde saçlı deri ve yüz bölgesinde soliter, verrüköz veya papiller lezyonlar şeklinde ortaya çıkar; lezyonlara sıklıkla sebase nevüs de eşlik edebilir. Histopatolojik incelemede, epidermisten dermise uzanan kistik invajinasyonlar ile lümene doğru uzantı gösteren çift katlı glandüler epitel yapısı karakteristiktir. Bu papiller yapıların dermal fibrovasküler korlarında plazma hücrelerinden zengin yoğun inflamatuar infiltrasyon bulunması SKAP için tipik bir tanısal kriterdir. İmmünohistokimyasal analizlerde luminal hücreler CEA, EMA ve çeşitli sitokeratinler ile pozitif reaksiyon gösterirken, genetik çalışmalarda BRAF ve HRAS mutasyonları öne çıkmaktadır. Ayırıcı tanıda hidradenoma papilliferum ve tübüler apokrin adenom gibi benzer morfolojideki lezyonlar dikkate alınmalıdır. Tedavisi cerrahi eksizyon ile gerçekleştirilir; nüks oranları son derece düşük olup bazal hücreli karsinom başta olmak üzere malign transformasyon gelişimi oldukça nadir bir durumdur.
Syringocystadenoma papilliferum (SCAP) is a rare benign cutaneous adnexal tumor thought to originate from apocrine or apo-eccrine glands. Most cases manifest at birth or before puberty as solitary, verrucous, or papillary lesions predominantly located on the scalp and face, frequently accompanied by an underlying nevus sebaceus. Histopathologically, it is characterized by cystic invaginations extending from the epidermis into the dermis, lined by a double-layered glandular epithelium projecting into the lumen. The presence of a dense inflammatory infiltrate rich in plasma cells within the dermal fibrovascular cores of these papillary structures represents a hallmark diagnostic feature of SCAP. In immunohistochemical analyses, luminal cells show positivity for CEA, EMA, and various cytokeratins, while genetic evaluations highlight BRAF and HRAS mutations. Differential diagnosis should carefully consider morphologically similar lesions such as hidradenoma papilliferum and tubular apocrine adenoma. The primary treatment is surgical excision; recurrence rates are extremely low, and malignant transformation, most notably coexistence with basal cell carcinoma, remains a very rare clinical event.
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