Uterus Sarkomları

Yazarlar

Şahin Yüksek

Özet

Uterin sarkomlar, uterusun düz kas ve bağ dokusundan kaynaklanan, tüm malign jinekolojik tümörlerin yaklaşık %1'ini oluşturan nadir ve agresif malignitelerdir. En sık 50-70 yaş arası postmenopozal dönemde görülen bu tümörlerin en yaygın alt tipi leiomyosarkomdur. Belirtileri non-spesifik olup pelvik kitle, ağrı ve vajinal kanamayı içerdiğinden benign miyomlarla kolayca karışabilir. Preoperatif dönemde laboratuvar veya görüntüleme yöntemleriyle (ultrason, MRG) benign-malign ayrımını kesin olarak yapmak zordur. Bu nedenle kesin tanı genellikle postoperatif histopatolojik incelemeyle konur. Standart cerrahi tedavisi, tümörün batın içine yayılımını önlemek amacıyla morselasyon yapılmadan, blok halinde total abdominal histerektomi ve bilateral salpingo-ooferektomidir. Erken evrelerde bile yüksek nüks ve metastaz riski taşıyan bu hastalıkta evre, en önemli prognostik faktördür. Adjuvan tedavide takip, radyoterapi, kemoterapi veya hormonal terapi seçenekleri nüks riskine ve histolojik alt tipe göre multidisipliner bir yaklaşımla belirlenir.

Uterine sarcomas are rare and aggressive malignancies originating from the smooth muscle and connective tissue elements of the uterus, accounting for approximately 1% of all malignant gynecological tumors. Most commonly diagnosed in postmenopausal women between the ages of 50 and 70, leiomyosarcoma represents the most frequent histological subtype. Because their clinical presentation is non-specific—often characterized by a pelvic mass, pain, and abnormal vaginal bleeding—they can easily be misdiagnosed as benign uterine fibroids. It remains highly challenging to differentiate benign leiomyomas from malignant sarcomas preoperatively using current laboratory tests or imaging modalities such as ultrasound and MRI; consequently, a definitive diagnosis is typically established through postoperative histopathological examination. The standard primary surgical management is a block total abdominal hysterectomy and bilateral salpingo-oophorectomy, where avoiding tumor morcellation is critical to prevent intraperitoneal dissemination. Disease stage serves as the most critical prognostic factor, as these tumors carry a high risk of local recurrence and distant metastasis, particularly to the lungs, even when detected at an early stage. Adjuvant treatment strategies, which include observation, radiotherapy, chemotherapy, or hormonal therapy, are tailored based on the specific histological subtype and recurrence risk through a strict multidisciplinary approach.

Referanslar

Bužinskienė D, Mikėnas S, Drąsutienė G, Mongirdas M. Uterine sarcoma: a clinical case and a literature review. Acta Med Litu. 2018;25(4):206-218. doi:10.6001/actamedica.v25i4.3931

Desar IME, Ottevanger PB, Benson C, van der Graaf WTA. Systemic treatment in adult uterine sarcomas. Crit Rev Oncol Hematol. 2018 Feb;122:10-20. doi: 10.1016/j.critrevonc.2017.12.009. Epub 2017 Dec 14. PMID: 29458779.

Roberts ME, Aynardi JT, Chu CS. Uterine leiomyosarcoma: A review of the literature and update on management options. Gynecol Oncol. 2018 Dec;151(3):562-572. doi: 10.1016/j.ygyno.2018.09.010. Epub 2018 Sep 21. PMID: 30244960.

Ricci S, Stone RL, Fader AN. Uterine leiomyosarcoma: Epidemiology, contemporary treatment strategies and the impact of uterine morcellation. Gynecol Oncol. 2017 Apr;145(1):208-216. doi: 10.1016/j.ygyno.2017.02.019. Epub 2017 Feb 13. PMID: 28209496.

D'Angelo E, Prat J. Uterine sarcomas: a review. Gynecol Oncol. 2010 Jan;116(1):131-9. doi: 10.1016/j.ygyno.2009.09.023. Epub 2009 Oct 23. PMID: 19853898.

Santos P, Cunha TM. Uterine sarcomas: clinical presentation and MRI features. Diagn Interv Radiol. 2015 Jan-Feb;21(1):4-9. doi: 10.5152/dir.2014.14053. PMID: 25347940; PMCID: PMC4463355.

D'Angelo E, Prat J. Uterine sarcomas: a review. Gynecol Oncol. 2010 Jan;116(1):131-9. doi: 10.1016/j.ygyno.2009.09.023. Epub 2009 Oct 23. PMID: 19853898.

El-Khalfaoui K, du Bois A, Heitz F, Kurzeder C, Sehouli J, Harter P. Current and future options in the management and treatment of uterine sarcoma. Ther Adv Med Oncol. 2014 Jan;6(1):21-8. doi: 10.1177/1758834013513314. PMID: 24381658; PMCID: PMC3866995.

Arend RC, Toboni MD, Montgomery AM, Burger RA, Olawaiye AB, Monk BJ, Herzog TJ. Systemic Treatment of Metastatic/Recurrent Uterine Leiomyosarcoma: A Changing Paradigm. Oncologist. 2018 Dec;23(12):1533-1545. doi: 10.1634/theoncologist.2018-0095. Epub 2018 Aug 23. PMID: 30139839; PMCID: PMC6292548.

Gelecek

28 Mart 2022

Lisans

Lisans