Primary Cardiac Leiomyosarcoma
2010; Lippincott Williams & Wilkins; Volume: 121; Issue: 21 Linguagem: Catalão
10.1161/circulationaha.109.879700
ISSN1524-4539
AutoresRuxandra Jurcuţ, Oana Savu, Bogdan A. Popescu, Anca Florian, Vlad Herlea, Horațiu Moldovan, Carmen Ginghină,
Tópico(s)Cardiac Structural Anomalies and Repair
ResumoHomeCirculationVol. 121, No. 21Primary Cardiac Leiomyosarcoma Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBPrimary Cardiac LeiomyosarcomaWhen Valvular Disease Becomes a Vascular Surgical Emergency Ruxandra Jurcut, MD, PhD, Oana Savu, MD, Bogdan A. Popescu, MD, PhD, Anca Florian, MD, Vlad Herlea, MD, Horatiu Moldovan, MD, PhD and Carmen Ginghina, MD, PhD Ruxandra JurcutRuxandra Jurcut From the Departments of Cardiology (R.J., O.S., B.A.P., A.F., C.G.), Pathology (V.H.), and Cardiac Surgery (H.M.), "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania. , Oana SavuOana Savu From the Departments of Cardiology (R.J., O.S., B.A.P., A.F., C.G.), Pathology (V.H.), and Cardiac Surgery (H.M.), "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania. , Bogdan A. PopescuBogdan A. Popescu From the Departments of Cardiology (R.J., O.S., B.A.P., A.F., C.G.), Pathology (V.H.), and Cardiac Surgery (H.M.), "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania. , Anca FlorianAnca Florian From the Departments of Cardiology (R.J., O.S., B.A.P., A.F., C.G.), Pathology (V.H.), and Cardiac Surgery (H.M.), "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania. , Vlad HerleaVlad Herlea From the Departments of Cardiology (R.J., O.S., B.A.P., A.F., C.G.), Pathology (V.H.), and Cardiac Surgery (H.M.), "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania. , Horatiu MoldovanHoratiu Moldovan From the Departments of Cardiology (R.J., O.S., B.A.P., A.F., C.G.), Pathology (V.H.), and Cardiac Surgery (H.M.), "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania. and Carmen GinghinaCarmen Ginghina From the Departments of Cardiology (R.J., O.S., B.A.P., A.F., C.G.), Pathology (V.H.), and Cardiac Surgery (H.M.), "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania. Originally published1 Jun 2010https://doi.org/10.1161/CIRCULATIONAHA.109.879700Circulation. 2010;121:e415–e418A 50-year-old previously healthy woman was referred to our department for suspected mitral valve endocarditis because of a 3-week history of fever, weight loss, and the echocardiographic discovery of a mitral valve mass. On admission, the patient was cachectic (body mass index of 18 kg/m2) and subfebrile (37.5°C) with dyspnea at rest, jugular vein distension, and tender liver enlargement. Her heart examination showed regular tachycardia (100 bpm), apical systolic murmur (3/6 degree), and diastolic rumble.The ECG showed sinus tachycardia (105 bpm) and signs of left atrial abnormality (wide, notched P wave measuring 140 ms; Figure 1). Chest x-ray revealed a cardiothoracic index of 0.6, with left atrial enlargement and interstitial edema (Figure 2). Download figureDownload PowerPointFigure 1. Twelve-lead ECG shows sinus rhythm, signs of left atrial abnormality (wide, notched P wave measuring 140 ms), and nonspecific ST-T changes.Download figureDownload PowerPointFigure 2. Posteroanterior chest x-ray reveals a cardiothoracic index of 0.6, signs of left atrial enlargement, and interstitial edema.Echocardiography showed an enlarged left atrium and a partially mobile mass on the mitral valve involving mainly the anterior leaflet and the anterolateral commissure (Figure 3A and 3B and Movies I and II in the online-only Data Supplement). This caused severe obstruction of the left ventricular inflow tract, with a mean transmitral gradient of 12 mm Hg and a functional valve area of 1 cm2 (Figure 4). Moderate mitral regurgitation was also noted. Transesophageal echocardiography confirmed the presence of a 22×11-mm highly mobile mass on the mitral valve oscillating between the left atrium and the left ventricle during the cardiac cycle (Figure 3C and 3D and Movie III in the online-only Data Supplement). No other intracardiac masses were detected. Download figureDownload PowerPointFigure 3. Mitral valve with mass (arrows) enclosing mainly the anterior leaflet and the anterolateral commissure in the transthoracic parasternal long-axis (A) and short-axis (B) views. Transesophageal echocardiography showing mass mobility between the left atrium (C; in systole) and the left ventricle (D; in diastole).Download figureDownload PowerPointFigure 4. Transmitral continuous wave Doppler flow. A, Mean gradient, 12 mm Hg. B, Mitral valve area (pressure–half-time method), 1 cm2.Shortly after admission, the patient presented a transient episode of left calf pain, paresthesia, and reduced power in the left lower limb that lasted for 30 minutes with complete recovery. Abdominal ultrasonography with color Doppler showed a filling defect at the level of aortic bifurcation and origin at the common iliac arteries (Figure 5). The episode was interpreted as a possible peripheral embolic event. Download figureDownload PowerPointFigure 5. Color Doppler evaluation of the terminal abdominal aorta showing anechogenous intraluminal material at bifurcation. CIA indicates common iliac artery; IVC, inferior vena cava.Coronary angiography showed normal coronary arteries, but injection into the abdominal aorta revealed a round filling defect at the level of the aortic bifurcation (Figure 6). Immediately afterward, echocardiography was repeated and showed the disappearance of the mobile part of the mass (Movie IV in the online-only Data Supplement), with a decrease in the transmitral mean gradient (Figure 7). Download figureDownload PowerPointFigure 6. Abdominal aortography showing a round filling defect at the level of aortic bifurcation with incomplete obstruction of both common iliac arteries.Download figureDownload PowerPointFigure 7. Two-dimensional aspect and transmitral continuous-wave Doppler flow before (A, C) and after (B, D) peripheral embolization showing disappearance of the mobile part of the mass (arrows) and decrease of the mean gradient from 12 to 9 mm Hg.Emergency surgical removal of both the intracardiac and intraaortic portions of the tumor was performed simultaneously under cardiopulmonary bypass. The mitral valve was excised with the anterolateral papillary muscle, which appeared on inspection to be invaded by the tumor, and a Sorin Bicarbon bileaflet mechanical mitral valve prosthesis (Sorin Biomedica, Saluggia, Italy) was implanted.Gross examination of the valve showed a 2-cm brown mass, confirming infiltration of the papillary muscle, and a similar macroscopic appearance of the arterial tumor emboli (Figure 8A). Histological examination of both the valvular mass and the intraaortic embolus showed that the tumor consisted of spindle cell proliferation, suggesting cardiac sarcoma (Figure 8B). Immunohistochemistry revealed nuclear polymorphism with atypical mitosis (with low mitotic index of 2 to 3 per 10 high-power fields) with positive staining for smooth muscle actin and vimentin (Figure 8C and 8D), confirming the diagnosis of leiomyosarcoma. Download figureDownload PowerPointFigure 8. A, Valvular tumor infiltrating the anterolateral papillary muscle and tumoral emboli extracted from terminal aorta (gross pathology). B, Histological appearance of cardiac sarcoma showing spindle cell proliferation (hematoxylin-eosin stain, ×400). Immunohistochemical stain of tumoral cells showing strong positivity for smooth muscular actin (C; ×100) and vimentin (D; ×200).A complete imaging workup was performed for a primary cause of the tumor (which was negative) and for secondary spread, which did not find any metastasis. Chemotherapy with docetaxel and gemcitabine was started, and 6 months after the initial diagnosis, the patient remained well and asymptomatic. Follow-up echocardiography at 6 months showed no cardiac tumor recurrence and normal prosthetic function.Differential diagnosis between infective endocarditis and cardiac tumor can be difficult if based on the clinical picture alone, but echocardiography can be helpful in differentiating a cardiac tumor from a vegetation. Primary cardiac leiomyosarcoma is a very rare tumor with a poor outcome.1 The most common site of origin for a primary cardiac sarcoma is the left atrium, whereas mitral valve origin is unusual (2 in a 15-case series).2 Complete resection of these tumors can rarely be achieved, but palliative surgery is usually undertaken because many patients present with mechanical obstruction. Adjunctive chemotherapy, radiation therapy, or both are sometimes used. However, the optimal protocol and treatment efficacy are unclear.3 The average survival without operation is 6 to 12 months; surgery extends survival to ≈24 months.4The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/121/21/e415/DC1.AcknowledgmentsWe would like to thank Ileana Arsenescu, MD, and Marian Albu, MD, for their assistance with vascular ultrasonography and arteriography imaging.DisclosuresNone.FootnotesCorrespondence to Ruxandra Jurcut, Department of Cardiology, "Prof Dr C.C. Iliescu" Institute of Emergency for Cardiovascular Diseases, Sos Fundeni No. 258, 022322 Bucharest, Romania. E-mail [email protected]References1 Antunes MJ, Vanderdonck KM, Andrade CM, Rebelo LS. Primary cardiac leiomyosarcomas. Ann Thorac Surg. 1991; 51: 999–1001.CrossrefMedlineGoogle Scholar2 Pessotto R, Silvestre G, Luciani GB, Anselmi M, Pasini F, Santini F, Mazzucco A. Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy. Int J Cardiol. 1997; 60: 91–94.CrossrefMedlineGoogle Scholar3 Burke A, Jeudy J, Virmani R. Cardiac tumours: an update. Heart. 2008; 94: 117–123.CrossrefMedlineGoogle Scholar4 Hattori Y, Iriyama T, Watanabe K, Negi K, Takeda I, Sugimura S. Rapidly growing primary cardiac leiomyosarcoma: report of a case. Surg Today. 2000; 30: 838–840.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Dumitru A, Aliuş C, Nica A, Antoniac I, Gheorghiță D and Grădinaru S (2020) Fatal outcome of gastric perforation due to infection with Sarcina spp. A case report, IDCases, 10.1016/j.idcr.2020.e00711, 19, (e00711), . Wang J, Cui L, Jiang T, Li Y and Wei Z (2015) Primary cardiac leiomyosarcoma: An analysis of clinical characteristics and outcome patterns, Asian Cardiovascular and Thoracic Annals, 10.1177/0218492315574197, 23:5, (623-630), Online publication date: 1-Jun-2015. Chu L, Johnson P, Halushka M and Fishman E (2012) Multidetector CT of the heart: spectrum of benign and malignant cardiac masses, Emergency Radiology, 10.1007/s10140-012-1055-y, 19:5, (415-428), Online publication date: 1-Oct-2012. Moldovan H, Sibișan A, Țigănașu R, Popescu B, Vasile G, Gheorghiță D, Zaharia O, Costache V, Guță A and Molnar A (2021) Superior Sinus Venosus Atrial Septal Defect with Partial Anomalous Pulmonary Venous Drainage—Minimally Invasive Approach—Case Report, Medicina, 10.3390/medicina57090984, 57:9, (984) Moldovan H, Sibisan A, Tiganasu R, Nechifor E, Gheorghita D, Zaharia O, Albu M, Popescu D, Molnar A, Craciun M and Scafa A (2021) Surgical Treatment in a High-Risk Pulmonary Embolism: Case Report, Medicina, 10.3390/medicina57070725, 57:7, (725) Moldovan H, Popescu B, Nechifor E, Badea A, Ciomaga I, Nica C, Zaharia O, Gheorghiță D, Broască M, Diaconu C, Parasca C, Chioncel O and Iliescu V (2022) Rare Cause of Severe Mitral Regurgitation after TAVI: Case Report and Literature Review, Medicina, 10.3390/medicina58040464, 58:4, (464) June 1, 2010Vol 121, Issue 21 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.109.879700PMID: 20516380 Originally publishedJune 1, 2010 PDF download Advertisement SubjectsCardiovascular SurgeryDiagnostic TestingEchocardiographyMyocardial Biology
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