Uterus Sarkomları
Özet
Uterus sarkomları, tüm uterin malignitelerinin %8’inden azını oluşturan, nadir görülen ve preoperatif tanısı sıklıkla net olmayan malign mezenkimal tümörlerdir. En yaygın histolojik alt tipleri leiomyosarkom (LMS) ve endometrial stromal sarkom (ESS) iken, undiferansiye uterin sarkomlar ve adenosarkomlar daha nadir izlenir. Sarkom vakalarının çoğunluğu sporadik olmakla birlikte, pelvik bölgeye radyoterapi öyküsü ve tamoksifen kullanımı risk faktörleri arasında yer almaktadır. Hastalığın evrelemesi histolojik alt tiplere göre değişiklik gösteren 2009 FIGO sistemine göre gerçekleştirilir. Erken evre LMS ve ESS vakalarında temel tedavi yaklaşımı total abdominal histerektomidir; hormonal duyarlılığı nedeniyle ESS olgularında bilateral salpingo-ooferektomi de sürece dahil edilir. Tüm alt tiplerde primer cerrahi sonrasında rezidü tümör kalmaması en önemli prognostik faktör olup, nüks riskini artırmamak adına uterus blok halinde çıkarılmalı ve morselasyondan kaçınılmalıdır. Güncel klinik veriler ışığında, erken evre hastalıkta adjuvan radyoterapi, kemoterapi veya hormonoterapinin sağkalım üzerinde kanıtlanmış standart bir faydası bulunmamaktadır. İleri evre, metastatik veya tekrarlayan hastalıklarda tedavi palyatif olup; tek ajan doksorubisin kemoterapisi, hedefe yönelik ajanlar (pazopanib, trabektedin) veya hormon reseptörü pozitif olgularda aromataz inhibitörleri gibi sistemik seçenekler yaşam kalitesini optimize etmek amacıyla kişiselleştirilerek tercih edilmektedir.
Uterine sarcomas are rare malignant mesenchymal tumors accounting for less than 8% of all uterine malignancies, often presenting without a definitive preoperative diagnosis. The most common histological subtypes are leiomyosarcoma (LMS) and endometrial stromal sarcoma (ESS), while undifferentiated uterine sarcomas and adenosarcomas are much less frequent. Although most cases are sporadic, a history of pelvic radiotherapy and tamoxifen use are identified as potential etiological risk faktors. Staging is performed according to the 2009 FIGO system, which varies based on the specific mesenchymal subtype. Total abdominal hysterectomy remains the cornerstone of standard treatment for early-stage LMS and ESS, with bilateral salpingo-oophorectomy typically added for ESS due to its distinct hormonal sensitivity. Achieving complete resection without residual tumor during primary surgery is the primary prognostic faktor; thus, the uterus must be removed en bloc, avoiding intraperitoneal morcellation to prevent disease recurrence. Currently, there is no proven survival benefit for routine adjuvant radiotherapy, chemotherapy, or hormone therapy in early-stage disease. In advanced, metastatic, or recurrent cases, treatment is strictly palliative; systemic options including single-agent doxorubicin, targeted therapies (pazopanib, trabectedin), or aromatase inhibitors for hormone receptor-positive tumors are personalized to maintain quality of life.
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