Mehmet Akif ÖNALAN, Ahmet DEMİRKAYA, Kemal BEHZATOĞLU, ERSİN EREK

Management of a primary cardiac leiomyosarcoma in a young woman

Cardiac leiomyosarcoma is an extremely rare tumor with a poor prognosis. An 18-year-old female patient was admitted to our clinic with a left atrial leiomyosarcoma extending to the right lower pulmonary veins. We performed complete tumor excision by the right anterolateral mini-thoracotomy approach using minimally invasive techniques. After pathological confirmation of the tumor, right lower lobectomy was performed with the same incision one week later to prevent recurrence. Although no tumor remnant was found in the lobectomy specimen, adjuvant chemotherapy was started. No recurrence was detected during the 12-month follow-up. In conclusion, the right submammarian minithoracotomy approach has the advantages of its less invasive nature and suitability for complete tumor resection with lobectomy.

Genç bir kadında primer kardiyak leiomyosarkom tedavisi

Kardiyak leiomyosarkom, kötü prognozlu, oldukça nadir görülen bir tümördür. Sağ alt pulmoner venlere uzanan sol atriyal leiomyosarkomu olan 18 yaşında bir kadın hasta kliniğimize başvurdu. Minimal invaziv teknikler kullanarak, sağ anterolateral mini torakotomi yaklaşımı ile tümörün tam eksizyonu gerçekletirildi. Tümörün patolojik olarak doğrulanmasının ardından bir hafta sonra aynı kesi ile nüksü önlemek için sağ alt lobektomi yapıldı. Lobektomi örneğinde tümör kalıntısı bulunmamasına rağmen, adjuvan kemoterapi başlandı. Hastanın 12 aylık takibinde nüks tespit edilmedi. Sonuç olarak, sağ submammarian minitorakotomi yaklaşımı, daha az invaziv olması ve lobektomi ile tümörün tam rezeksiyonu için uygun olması avantajlarına sahiptir.

PDF

___

1. Patel J, Sheppard MN. Pathological study of primary cardiac and pericardial tumours in a specialist UK Centre: Surgical and autopsy series. Cardiovasc Pathol 2010;19:343-52.
2. Gukop P, Frassetto G, Karapanagiotidis G, Chandrasekaran V. Leiomyosarcoma of pulmonary vein presenting as left atrial mass: An outline of management principles. Case Rep Cardiol 2012;2012:396319.
3. Wang JG, Cui L, Jiang T, Li YJ, Wei ZM. Primary cardiac leiomyosarcoma: An analysis of clinical characteristics and outcome patterns. Asian Cardiovasc Thorac Ann 2015;23:623-30.
4. Morin JE, Rahal DP, Hüttner I. Myxoid leiomyosarcoma of the left atrium: A rare malignancy of the heart and its comparison with atrial myxoma. Can J Cardiol 2001;17:331-6.
5. Patel SM, Kadakia KC, Maleszewski JJ, Marks RS. Straight to the heart: Pulmonary vein leiomyosarcoma. Am J Med 2013;126:117-9.
6. Elbardissi AW, Dearani JA, Daly RC, Mullany CJ, Orszulak TA, Puga FJ, et al. Survival after resection of primary cardiac tumors: A 48-year experience. Circulation 2008;118(14 Suppl):S7-15.