Flavia COCCONCELLİ, Flippo BENASSİ, Matteo SCAPİNELLİ, ROBERTO PARRAVİCİNİ

Right atrial angiosarcoma mimicking a myxoma: from a wrong diagnosis to a difficult decision

Bu yazıda, kardiyak miksoma tanısı konan büyük bir sağ atriyal kitlenin çıkarılması için cerrahi tedavi uygulanan 27 yaşında erkek hasta sunuldu. Kitlenin oldukça büyük olması nedeniyle ameliyat sırasında birkaç biyopsi uygulandı. Anjiyosarkom tanısı kondu, dolayısıyla cerrahi eksizyon tek çareydi. Altı ay sonra bilgisayarlı tomografi sonucunda hemorajik serebral lezyonların olduğu görüldü. Hasta ameliyattan 10 ay sonra kaybedildi. Primer anjiyosarkom için en iyi tedavi yöntemi erken ve tam bir cerrahi eksizyon olacaktır. Ne yazık ki sıklıkla hayati kardiyak yapıların önemli bir bölümünün de tutulumu görülür, bu durumda ortotopik kardiyak transplantasyon düşünülebilir.

Miksomayı andıran sağ atriyal anjiyosarkom: yanlış bir tanıdan zor bir karara

The case described deals with a 27-year old man who underwent surgical approach to remove a large right atrial mass diagnosed as cardiac myxoma. During the operation several biopsies were performed because of its gross aspect. An angiosarcoma was diagnosed, thus, a surgical excision was the only possible procedure. Six months later, a computed tomography scan revealed the presence of substitutive and hemorrhagic cerebral lesions. The patient expired 10 months after surgery. The best therapy for primary angiosarcoma would have been an early and complete surgical excision. Unfortunately, often an important involvement of vital cardiac structures is present; in this case an orthotopic cardiac transplantation could be considered.

PDF

___

1. Brandt RR, Arnold R, Bohle RM, Dill T, Hanım CW. Cardiac angiosarcoma: case report and review of the literature. Z Kardiol 2005;94:824-8.
2. Grandmougin D, Fayad G, Decoene C, Pol A, Warembourg H. Total orthotopic heart transplantation for primary cardiac rhabdomyosarcoma: factors influencing long-term survival. Ann Thorac Surg 2001;71:1438-41.
3. Kodali D, Seetharaman K. Primary cardiac angiosarcoma. Sarcoma 2006;2006:39130.
4. Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardiothorac Surg 1997;12:730-8.
5. Uberfuhr P, Meiser B, Fuchs A, Schulze C, Reichenspurner H, Faik M, et al. Heart transplantation: an approach to treating primary cardiac sarcoma? J Heart Lung Transplant 2002; 21:1135-9.
6. Putnam JB Jr, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg 1991;51:906-10.
7. Kurian KC, Weisshaar D, Parekh H, Berry GJ, Reitz B. Primary cardiac angiosarcoma: case report and review of the literature. Cardiovasc Pathol 2006;15:110-2.
8. Nakamichi T, Fukuda T, Suzuki T, Kaneko T, Morikawa Y. Primary cardiac angiosarcoma: 53 months' survival after multidisciplinary therapy. Ann Thorac Surg 1997;63:1160-1.
9. Sorlie D, Myhre ES, Stalsberg H. Angiosarcoma of the heart. Unusual presentation and survival after treatment. Br Heart J 1984;51:94-7.
10. Percy RF, Perryman RA, Amornmarn R, Lammert N, Conetta DA, Bass TA, Miller AB. Prolonged survival in a patient with primary angiosarcoma of the heart. Am Heart J 1987;113:1228-30.
11. Aravot DJ, Banner NR, Madden B, Aranki S, Khaghani A, Fitzgerald M, et al. Primary cardiac tumours-is there a place for cardiac transplantation? Eur J Cardiothorac Surg 1989; 3:521-4.