Spiradenom
Özet
Spiradenom, klinik olarak baş-boyun bölgesinde ve gövdenin üst yarısında paroksismal ağrı veya hassasiyetle seyreden, yavaş büyüyen soliter nodüllerle karakterize nadir ve benign bir deri eki tümörüdür. Tarihsel olarak ekrin diferansiyasyon gösterdiği varsayılsa da, günümüzde tümör hücrelerinin kıl folikülü çıkıntısı kök hücre belirteci olan CD200 için pozitiflik göstermesi, lezyonun ter bezlerinden ziyade follikülosebasöz-apokrin kökenli olduğunu güçlü bir şekilde düşündürmektedir. Histopatolojik olarak dermiste yerleşen, ince bir fibröz kapsülle çevrili bazofilik nodüller, periferde küçük hiperkromatik hücreler ve merkezde oval veziküler çekirdekli büyük hücreler olmak üzere iki tip hücre popülasyonu ile belirgin duktal ve vasküler yapılar içerir. İmmünhistokimyasal incelemelerde CK5/6, CK7, CK8, CK18 ve EMA pozitifliği izlenirken; miyoepitelyal hücrelerde SMA, p63 ve S100 antikorları ile reaksiyon saptanır. Otozomal dominant geçişli Brooke-Spiegler Sendromu parçası olarak çoklu lezyonlar şeklinde de görülebilen bu neoplazide, kromozom 16q12.1 üzerindeki CYLD tümör baskılayıcı gen mutasyonları patogenezde kritik rol oynar. Ayırıcı tanıda anjiyoleiomyom, glomus tümörü ve metastatik kutanöz adenokarsinomlar titizlikle elenmelidir. Nadir de olsa malign dönüşüm riski ve yetersiz cerrahi sınırlardan kaynaklanan nüks olasılığı bulunduğundan, tam cerrahi eksizyon ve sonrasında uzun dönemli klinik takip en güvenilir tedavi yaklaşımıdır.
Spiradenoma is a rare, benign skin adnexal neoplasm first characterized in 1956, typically presenting as a slow-growing, solitary intradermal nodule that causes paroxysmal pain or tenderness, most frequently arising in the head, neck, and upper trunk regions. Although historically presumed to be of eccrine origin, recent immunohistochemical investigations demonstrating tumor cell positivity for the hair follicle bulge stem cell marker CD200 strongly indicate a folliculosebaceous-apocrine lineage rather than a true sweat gland origin. Histopathologically, the lesion manifests as well-demarcated basophilic dermal nodules enclosed by a fibrous capsule, composed of a distinct dual cell population: small hyperchromatic cells at the periphery and larger pale cells with vesicular nuclei centrally, often showing clear ductal differentiation and dense vascularization. Immunohistochemical staining characteristically reveals cytoplasmic reactivity for CK5/6, CK7, CK8, and CK18, alongside SMA, p63, and S100 expression within the myoepithelial components. In hereditary cases linked to Brooke-Spiegler Syndrome, mutations or loss of heterozygosity in the CYLD tumor suppressor gene located on chromosome 16q12.1 drive the pathogenesis. Differential diagnosis includes distinguishing it from other painful or highly vascularized tumors like angioleiomyoma, glomus tumor, or metastatic adenocarcinoma. Because of the potential for recurrence associated with inadequate surgical margins and the rare risk of malignant transformation, wide local surgical excision combined with routine clinical monitoring constitutes the optimal management strategy.
Referanslar
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