Prolifere Pilar Tümör
Özet
Prolifere pilar tümör (PPT), kıl folikülünün dış kök kılıfından köken alan, nadir rastlanan ve çoğunlukla benign karakterde olmakla birlikte yerel agresif veya malign özellikler de sergileyebilen bir tümör modelidir. Genellikle kırk yaş üzerindeki kadın hastalarda, belirgin şekilde saçlı deri yerleşimi gösteren, yavaş büyüme eğilimindeki ekzofitik ve ülserize olabilen kitleler olarak klinik yansıma bulur. Bilgisayarlı tomografi gibi radyolojik görüntülemelerde kaba kalsifikasyonlar ve kompleks subkutan yapılar şeklinde izlenen neoplazi, makroskopik olarak 2 ila 25 santimetre boyutlarında, sert kıvamlı ve lobüle kontürlüdür. Histopatolojik açıdan, trikilemmal kistlerden farklı olarak lümene doğru prolifere olan anastomozlaşan epitelyal hücreler ve karakteristik trikilemmal keratinizasyon alanları ile ayırt edilir. Ayırıcı tanısında epidermoid kist, pilomatriksoma ve özellikle skuamöz hücreli karsinom (SHK) kritik önem taşır; morfolojik bütünlük ve CD34 pozitifliği SHK ile ayrımda klinisyene rehberlik eder. Genetik olarak tümör baskılayıcı TP53 mutasyonları agresif dönüşüm süreçleriyle ilişkilendirilmiştir. Tedavide esas seçenek küratif basit cerrahi eksizyon olup, yüksek nüks ve metastaz potansiyeline sahip malign varyantlarında bir santimetrelik temiz cerrahi sınırların sağlanması, gerektiğinde ise radyoterapi ve kemoterapi gibi multimodalite yaklaşımların tercih edilmesi optimal klinik başarı için elzemdir.
Proliferating pilar tumor (PPT) is a rare neoplasm originating from the outer root sheath of the hair follicle, which is predominantly benign but can exhibit locally aggressive or malignant behavior. It most frequently occurs in women over the age of 40, with more than 90% of cases localized on the scalp as slow-growing, exophytic, and sometimes ulcerated masses. On radiological imaging such as computed tomography, it presents as complex subcutaneous solid or cystic masses containing coarse calcifications. Macroscopically, the lesions range from 2 to 25 cm, showing a firm consistency and lobulated contours. Histopathologically, PPT is characterized by multi-layered, anastomosing epithelial cells with abundant eosinophilic cytoplasm proliferating into the cyst lumen, accompanied by distinctive trichilemmal keratinization. Differential diagnosis includes epidermoid cysts, pilomatricomas, and particularly squamous cell carcinoma (SCC), where the presence of trichilemmal remnants and CD34 immunoreactivity supports PPT differentiation. Genetically, TP53 tumor suppressor gene mutations are implicated in malignant transformation. Although simple surgical excision is the primary curative treatment, malignant variants showing destructive invasion, high mitotic activity, and metastatic potential require wide local excision with a 1 cm clear margin, occasionally supplemented by radiotherapy or chemotherapy to optimize long-term prognosis.
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