Siringom
Özet
Siringom, intradermal ekrin ter kanalının düz segmentinden köken alan, genellikle sporadik seyirli, iyi huylu bir deri eki tümörüdür. Klinik olarak çoğunlukla ergenlik veya erken erişkinlik dönemindeki kadınlarda, göz kapakları ve üst yanakta simetrik yerleşimli, 1-2 mm boyutlarında, asemptomatik ve deri renginde multipl papüller olarak izlenir. Bununla birlikte dev lezyonlar, plak veya milia benzeri varyantlar ile genital bölge gibi farklı lokalizasyonlar da bildirilmiştir. Histopatolojik olarak yüzeyel dermiste, fibröz stroma içerisinde dağılmış, iki katlı küboidal epitel ile döşeli, tipik "virgül benzeri" kuyruğu olan küçük duktal yapılar karakteristiktir. İmmünohistokimyasal olarak EKH-6 ve çeşitli sitokeratinler ile pozitif boyanma gösterirken, progesteron reseptör ekspresyonu hormonal kontrolü düşündürmektedir. Elektron mikroskopisinde duktal lümenlerin mikrovilluslu hücreler tarafından kaplandığı ve intrasitoplazmik boşlukların birleşmesiyle oluştuğu görülür. Ayırıcı tanıda siringoid ekrin karsinom ve mikrokistik adneksiyal karsinom gibi malign antitelerden ayrımı, atipi, infiltrasyon ve perinöral invazyon yokluğu ile yapılır. Benign karakterde olduklarından tedavi gerektirmezler; ancak kozmetik kaygılarla eksizyon, kriyoterapi veya lazer ablasyon gibi ablatif yöntemler başarıyla uygulanabilir.
Syringoma is a benign, usually sporadic cutaneous adnexal tumor originating from the straight segment of the intradermal eccrine sweat duct. Clinically, it presents predominantly in females during adolescence or early adulthood as multiple, symmetrical, asymptomatic, skin-colored to yellowish papules, 1-2 mm in size, typically localized on the eyelids and upper cheeks. However, various presentations including giant solitary lesions, plaque-like, or milia-like variants can occur across diverse sites like the vulva or scalp. Histopathologically, it is characterized by numerous small ductal structures embedded in a fibrous stroma within the superficial dermis, lined by a double layer of cuboidal cells, often displaying a distinctive "comma-like" tail. Immunohistochemically, syringomas express EKH-6 and specific cytokeratins, alongside progesterone receptors, suggesting hormonal influence. Electron microscopy reveals ductal lumens lined by cells with microvilli, formed through the coalescence of intracytoplasmic vacuoles mimicking embryonic eccrine duct development. Differentiation from rare malignant counterparts, such as syringoid eccrine carcinoma and microcystic adnexal carcinoma, is critical and based on the absence of nuclear atypia, deep infiltration, and perineural invasion. Being entirely benign, treatment is unnecessary except for cosmetic purposes, where modalities like excision, cryotherapy, or laser ablation are successfully utilized.
Referanslar
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