Hidradenoma Papilliferum
Özet
Hidradenoma papilliferum (HP), anogenital meme benzeri gland kaynaklı, genellikle orta yaş kadınların vulva ve perianal bölgelerinde yerleşen benign bir deri eki tümörüdür. Klinik olarak 1 cm'den küçük, düzgün sınırlı, mobil, soliter papül veya nodül şeklinde izlenir. Histopatolojik açıdan, dermis yerleşimli bu lezyon çift katlı glandüler epitel ile döşeli, dallanan ve anastomozlaşan tübüler yapılar ile labirent benzeri papiller bir görünüm sergiler. Luminal kısımda dekapitasyon sekresyonu gösteren yüksek kolumnar hücreler pozitif EMA, CK7 ve ER ekspresyonu sunarken; bazaldeki myoepitel tabakası S100 ve SMA ile boyanır. Son genetik araştırmalar, tümörün patogenezinde PIK3CA/AKT sinyal yolağı mutasyonlarının baskın olduğunu ortaya koymaktadır. Ayırıcı tanıda siringokistadenoma papilliferum ve adenokarsinom gibi patolojiler değerlendirilir; ancak belirgin pleomorfizmin olmaması ve korunan myoepitel tabakası tümörün benign doğasını doğrular. Malign transformasyon ve nüks oranları oldukça düşüktür. Tedavide basit cerrahi eksizyon yeterli ve küratiftir.
Hidradenoma papilliferum (HP) is a benign cutaneous adnexal tumor originating from anogenital mammary-like glands, predominantly affecting the vulva and perianal regions of middle-aged women. Clinically, it presents as a smooth, mobile, solitary papule or nodule, typically measuring less than 1 cm. Histopathologically, this dermal lesion features a well-circumscribed, labyrinth-like pattern composed of branching and anastomosing tubular structures lined by a double-layered glandular epithelium. The luminal aspect consists of columnar cells with decapitation secretion that express EMA, CK7, and ER, whereas the basal myoepithelial layer stains positive for S100 and SMA. Recent molecular investigations reveal frequent mutations associated with the PIK3CA/AKT signaling pathway. Differential diagnosis includes syringocystadenoma papilliferum and adenocarcinoma; however, the absence of marked pleomorphism and the preservation of the myoepithelial layer confirm its benign nature. Malignant transformation and recurrence rates are exceptionally low. Complete simple surgical excision remains the standard curative treatment.
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