Kutanöz Apokrin Karsinom
Özet
Kutanöz apokrin karsinom, apokrin bezlerin malignitesi sonucu gelişen ve oldukça nadir görülen bir deri eki tümörüdür. Çoğunlukla koltuk altı (aksilla) bölgesinde yerleşmekle birlikte baş, boyun, vulva ve meme başı gibi apokrin bezlerin yer aldığı farklı anatomik lokalizasyonlarda da ortaya çıkabilmektedir. Genellikle yedinci dekatta, yavaş büyüyen ve nodüler karakterde lezyonlar şeklinde klinik prezentasyon gösterir. Histopatolojik olarak incelendiğinde derin dermis ile subkutan dokuda kapsülsüz, tübüler, papiller ya da solid alanlar oluşturan kompleks glandüler yapılar izlenir. Tümör hücreleri, bol eozinofilik ve granüler sitoplazmaya sahip olup karakteristik dekapitasyon salgısı barındırır. İmmünhistokimyasal profilde CK7, GCDFP-15, GATA3 ve androjen reseptörü pozitifliği belirginken, ekrin tümörlerin aksine p63 ekspresyonu genellikle negatif saptanır. Ayırıcı tanıda memenin primer veya metastatik apokrin karsinomlarından ayırt edilmesi kritik bir önem taşır. Bu ayrımda lezyon çevresinde normal apokrin glandların varlığı ve sitoplazmik demir granülleri kutanöz orijini destekleyen en önemli histolojik bulgulardır. Bölgesel lenf nodu ve visseral organ metastazı yapabilen bu lokal invaziv tümörlerde, histolojik diferansiyasyon derecesi ile sağ kalım oranı arasında doğrudan bir ilişki bulunmaktadır.
Primary cutaneous apocrine carcinoma is an extremely rare skin adnexal neoplasm arising from the malignant transformation of apocrine sweat glands. Although the axilla is the most common site of presentation, it can also develop in other anatomical locations containing apocrine glands, including the head, neck, vulva, and nipple. It characteristically manifests in the seventh decade of life as slow-growing, solitary or multiple nodules. Histopathologically, these unencapsulated tumors reside in the deep dermis and subcutaneous tissue, showing complex glandular patterns with tubular, papillary, or solid structures. The tumor cells exhibit abundant eosinophilic granular cytoplasm and variable decapitation secretion. Immunohistochemically, the neoplastic cells express CK7, GCDFP-15, GATA3, and androgen receptors, while typically lacking p63 expression. Differentiating this primary cutaneous tumor from metastatic breast apocrine carcinoma or ectopic breast tissue carcinoma is highly challenging but crucial. The presence of adjacent normal apocrine glands and intracytoplasmic iron granules strongly supports a primary cutaneous origin. These locally invasive neoplasms can metastasize to regional lymph nodes and visceral organs, where tumor differentiation correlates with overall patient survival rates.
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