Hidradenokarsinom
Özet
Hidradenokarsinom (HAK), ekrin veya apokrin bezlerin intradermal duktusundan köken alan, oldukça nadir ve agresif bir malign deri eki tümörüdür. Tüm malign ekrin tümörlerin yaklaşık %6'sını oluşturan bu hastalık, sıklıkla 50-70 yaş aralığındaki bireylerde ve baş-boyun bölgesinde yavaş büyüyen, soliter, subkutan bir kitle olarak klinik verir. Histopatolojik olarak dermiste yerleşen tümör; belirgin pleomorfizm, yüksek mitotik aktivite (>4/10 BBA), atipik mitoz ve komedonekroz alanları ile karakterizedir ve berrak veya skuamoid hücre morfolojisi sıklıkla izlenir. Tanıda ve iyi huylu hidradenomlardan ayrımında yüksek Ki-67 proliferasyon indeksi, p53 yaygın pozitifliği ile EMA ve CEA duktal boyanmaları yardımcı olmaktadır. Yüksek nüks (%50-75) ve erken dönem lenf nodu ile uzak organ metastazı riskine sahip olan HAK'ta 5 yıllık sağkalım oranı %30 civarındadır. Tedavinin temelini rezeksiyon öncesi sentinel lenf nodu biyopsisinin de eşlik ettiği geniş cerrahi eksizyon oluşturur; cerrahi sınırların yetersiz olduğu ya da metastatik durumlarda radyoterapi ile 5-florourasil ve kapesitabin bazlı kemoterapi şemaları tedaviye eklenir.
Hidradenocarcinoma (HAC) is a highly rare and aggressive malignant skin adnexal tumor originating from the intradermal duct of eccrine or apocrine glands. Accounting for approximately 6% of all malignant eccrine tumors, it frequently presents in individuals aged 50-70 as a slow-growing, solitary, subcutaneous mass, predominantly located in the head and neck region. Histopathologically localized within the dermis, the tumor is characterized by prominent pleomorphism, high mitotic activity (>4/10 HPF), atypical mitosis, and comedonecrosis, with clear or squamoid cell morphologies being frequently observed. High Ki-67 proliferation index, diffuse p53 positivity, and luminal EMA and CEA ductal expressions assist in diagnosis and differentiation from benign hydradenomas. Exhibiting a high recurrence rate of 50-75% and substantial risk for early lymph node and distant metastases, the 5-year survival rate remains around 30%. Wide surgical excision, typically preceded by sentinel lymph node biopsy, forms the mainstay of treatment; radiation and chemotherapeutic regimens utilizing 5-fluorouracil and capecitabine are integrated in cases of positive surgical margins or advanced metastatic disease.
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