Meme Sarkomları
Özet
Primer meme sarkomları, tüm meme tümörlerinin %1’inden, tüm sarkomların ise %5’inden daha azını oluşturan nadir mezenkimal tümörlerdir. En sık 50-60 yaşlarında görülen bu tümörlerin çoğunluğu, radyoterapi uygulamalarına ikincil olarak gelişir. Risk faktörleri arasında dış ışın radyasyonu, kronik lenfödem ve kalıtsal sendromlar yer alır. Genellikle yüksek dereceli olup hızla büyüyen tek kitleler şeklinde ortaya çıkarlar. Tanıda fizik muayene, mamografi, sonografi ve MR kullanılmakla birlikte, kesin teşhis için histopatolojik ve immünohistokimyasal analiz kritik önem taşır; en sık anjiyosarkom alt tipi görülür. Bu tümörler yüksek nüks riskine sahip olup, hematojen yolla çoğunlukla akciğer, kemik iliği ve karaciğere metastaz yaparlar. Tedavide multidisipliner bir yaklaşım şarttır ve tek iyileştirici yöntem negatif cerrahi sınırlı rezeksiyondur; lenf nodu diseksiyonu ise düşük metastaz oranı nedeniyle önerilmez. Tümör boyutu ve cerrahi sınırın temizliği mortaliteyi belirleyen en önemli faktörlerdir. Genellikle kemoterapiye zayıf yanıt verirler ve adjuvan radyoterapinin faydaları konusunda literatürde henüz tam bir fikir birliği bulunmamaktadır.
Primary breast sarcomas are rare mesenchymal tumors that account for less than 1% of all breast tumors and fewer than 5% of all sarcomas, predominantly peaking between the ages of 50 and 60, with most cases occurring secondarily to radiation therapy. Although risk factors include external beam radiation, chronic lymphedema, and genetic syndromes, these tumors present as rapidly growing solitary masses and differ from epithelial breast cancers as they mimic connective tissue. Diagnosis utilizes mammography, ultrasound, and MRI, but histopathological confirmation with immunohistochemical panels is essential, with angiosarcoma, malignant fibrous histiocytoma, and stromal sarcoma being the most common subtypes. Characterized by high recurrence rates and poor prognosis, they metastasize hematogenously to the lungs, bone marrow, and liver. Surgical resection with negative margins remains the only curative approach, whereas lymph node dissection is not recommended due to low nodal involvement. Ultimately, tumor size and clear surgical margins are the primary determinants of survival, while the roles of adjuvant chemotherapy and radiotherapy remain limited and lack consensus in current literature due to weak response rates.
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