Anal Paget Hastalığı
Özet
Anal Paget Hastalığı (APH), apokrin bezlerin bulunduğu bölgelerde ortaya çıkan nadir bir intraepitelyal adenokarsinomdur. Meme dışı Paget hastalığının bir formu olan APH, genellikle ileri yaştaki bireylerde görülür ve internal malignitelerle olan ilişkisine göre primer veya sekonder olarak sınıflandırılır; primer olgular epidermal hücrelerden köken alırken, sekonder olgular genellikle altta yatan kolorektal veya genitoüriner bir kanserin yayılımı sonucu oluşur. Klinik olarak patognomonik bir bulgusu olmayan hastalık, sıklıkla kronik dermatit veya egzama ile karıştırılan şiddetli kaşıntı ve eritematöz plaklarla karakterizedir. Tanı sürecinde tam kat cilt biyopsisi ve immünohistokimyasal boyamalar kritik öneme sahiptir; CK7, CK20 ve GCDFP-15 gibi belirteçler primer ve sekonder ayrımında kullanılır. Tedavi yaklaşımı genellikle geniş cerrahi eksizyonu kapsar, ancak nüks oranlarının %30 civarında olması nedeniyle Mohs mikrografik cerrahisi gibi doku koruyucu yöntemler ve radyoterapi de değerlendirilebilir. Prognozu genel olarak iyi olsa da, derin dermal invazyon ve uzak metastaz varlığı sağkalımı olumsuz etkilemektedir.
Anal Paget’s Disease (APD) is a rare intraepithelial adenocarcinoma occurring in areas rich in apocrine glands. As a form of extramammary Paget’s disease, APD typically affects elderly individuals and is classified as primary or secondary based on its association with internal malignancies; primary cases originate from epidermal cells, while secondary cases result from the spread of underlying colorectal or genitourinary cancers. Lacking pathognomonic findings, the disease is clinically characterized by severe itching and erythematous plaques often mistaken for chronic dermatitis or eczema. Full-thickness skin biopsy and immunohistochemical staining are critical for diagnosis, utilizing markers such as CK7, CK20, and GCDFP-15 to differentiate between primary and secondary forms. The treatment approach generally involves wide surgical excision, though tissue-sparing techniques like Mohs micrographic surgery and radiotherapy are also considered due to recurrence rates around 30%. While the prognosis is generally favorable, factors such as deep dermal invasion and distant metastasis negatively impact survival outcomes.
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