Mikrokistik Adneksiyel Karsinom
Özet
Mikrokistik Adneksiyel Karsinom (MAK), genellikle baş ve boyun bölgesinde ortaya çıkan, yavaş büyüyen ancak derin lokal invazyon ve perinöral yayılım eğilimi gösteren nadir bir malign deri tümörüdür. Epidemiyolojik olarak orta ve ileri yaştaki beyaz tenli bireylerde ve hafif kadın baskınlığıyla görülen bu neoplazmın insidansı oldukça düşüktür. Ultraviyole, iyonize radyasyon ve immünsupresyon potansiyel risk faktörleri arasında sayılsa da kesin etiyolojisi net değildir. Klinik olarak telenjiektazili, belirsiz sınırlı beyaz-sarı papül veya plaklar şeklinde prezante olur ve asemptomatik seyredebilmekle birlikte perinöral tutuluma bağlı lokal ağrı veya paresteziye yol açabilir. Histopatolojik açıdan desmoplastik trikoepitelyoma veya morfeaform bazal hücreli karsinom (BCC) gibi maligniteleri taklit edebildiğinden tanısal zorluklar taşır; bu nedenle derin punch veya tıraş biyopsisi gereklidir. Ayırıcı tanıda CEA, CK7 ve CK15 pozitifliği ile Ber-EP4 negatifliği gibi immünhistokimyasal belirteçlerden yararlanılır. Tedavide, nüks oranlarını en aza indiren Mohs mikrografik cerrahisi altın standart kabul edilir. Cerrahi sınır pozitifliği olan ya da cerrahiye uygun olmayan olgularda radyoterapi ve sınırlı sayıdaki metastatik vakada kemoterapi birer seçenek olsa da etkinlikleri kısıtlıdır. Uzak metastaz nadir görülse de yıllar sonra bile gecikmiş lokal nüks riski bulunduğundan uzun süreli takip şarttır.
Microcystic Adnexal Carcinoma (MAC) is a rare, slow-growing malignant skin tumor primarily arising in the head and neck region, characterized by deep local invasion and high perineural propagation. Epidemiologically, it shows a low incidence, predominantly affecting middle-aged or elderly fair-skinned individuals with a slight female predominance. Although ultraviolet light, ionizing radiation, and immunosuppression are implicated as potential risk factors, the definitive etiology remains unclear. Clinically presenting as inconspicuous, ill-defined whitish-yellow papules or plaques with or without telangiectasia, it is mostly asymptomatic but can induce localized pain or paresthesia due to perineural involvement. Histopathologically, it mimics benign or malignant lesions like desmoplastic trichoepithelioma or morpheafrom basal cell carcinoma (BCC), necessitating deep punch or shave biopsies for correct diagnosis. Immunohistochemical markers like CEA, CK7, and CK15 positivity alongside Ber-EP4 negativity facilitate differentiation. Surgical intervention via Mohs micrographic surgery represents the primary treatment modality due to superior margin control and lower recurrence rates. Radiotherapy for positive margins and systemic chemotherapy for rare metastatic cases offer limited efficacy. While distant metastasis remains exceedingly rare, prolonged clinical follow-up is imperative due to the high propensity for delayed local recurrences spanning decades.
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