Scand J Thorac Cardiovasc Surg 1987;21(3):277-9
Extensive surgery for left atrial leiomyosarcoma diagnosed by echocardiography. A case report.
Schmekel B, Landelius J, Aberg T, Enghoff E.
Department of Clinical Physiology, University Hospital, Uppsala, Sweden.
A case of malignant leiomyosarcoma presenting as a left atrial cardiac tumor is described and its echocardiographic features are discussed. Tumor masses invading the atrial walls were extensively resected and the atrial wall was reconstructed with two patches of bovine pericardium.
Heart 2001 Jul;86(1):E2
Leiomyosarcoma of the right ventricle extending into the pulmonary trunk.
Willaert W, Claessens P, Vanderheyden M.
Department of Cardiology, Imelda Ziekenhuis, Bonheiden, B-2820, Belgium.
Primary leiomyosarcomas of the heart, particularly those affecting the right ventricle, are uncommon. A 70 year old woman presenting with the symptoms of progressive exertional dyspnoea and left sided pleuritic pain is presented. A leiomyosarcoma was diagnosed that originated in the right lateral ventricle wall, causing pulmonary outflow obstruction. Pathological analysis showed a neoplasm with a myxoid stroma, high mitotic activity, and nuclei expressing atypia. Immunohistochemical staining was found positive for vimentin and desmin. Seven months after complete surgical resection the tumour relapsed. This case illustrates the poor outcome, high relapse rate, and inefficiency of treatment associated with primary cardiac leiomyosarcomas. The current literature regarding incidence, diagnostic techniques, treatment strategies, and survival rates of this rare but terminal disease is reviewed.
Thorac Cardiovasc Surg 2002 Feb;50(1):62-3
Redo- extirpation of a cardiac leiomyosarcoma to avoid transplantation.
Wippermann J, Albers JM, Brandes H, Wahlers T.
Department of Cardiac, Thoracic and Vascular Surgery, Universty Hospital Jena, Germany. Jens.Wippermann@med.uni-jena.de
Cardiac leiomyosarcoma is a very rare entity that is found in less than 0.2 % of all cardiac tumors. At the time of primary diagnosis, it often shows advanced local invasion or may even be metastasized. Thus, complete resection can not easily be achieved. Cardiac transplantation has been reported as a therapeutic option. Here, we report on a case of a leiomyosarcoma reoccurrence arising 2 years after initial surgery. We performed a radical redo-extirpation without the necessity of transplantation.
Jpn J Thorac Cardiovasc Surg 1998 Dec;46(12):1339-44
Primary leiomyosarcoma of the heart, report of a case and review of the literature
Myojin K, Ishibashi Y, Ishii K, Murakami T, Itoh M.
Division of Cardiovascular Surgery, National Sapporo Hospital, Hokkaido, Japan.
Primary cardiac neoplasms are unusual and are found in only about 0.0017% of autopsies. Approximately 25% of primary cardiac neoplasms are malignant. A case of primary leiomyosarcoma arising from the right ventricle and the pulmonary artery is reported. A 37-year-old man had sudden dyspnea and chest pain. Angiocardiography and MRI revealed moving masses throughout the right ventricle and the pulmonary arteries. An emergent surgery was performed. The multiple tumors were observed and incomplete resection was done. The patient was survived with the aid of a veno-arterial bypass as an assisted circulation on the first postoperative day. However, he gradually became deteriorated again and died 8 days after the operation due to the right ventricular failure. Eleven cases of the primary cardiac leiomyosarcoma including our case in the Japanese literature were reviewed briefly.
J Pediatr Hematol Oncol 1996 Aug;18(3):314-7
Successful treatment of a primary cardiac leiomyosarcoma with ifosfamide and etoposide.
Han P, Drachtman RA, Amenta P, Ettinger LJ.
Department of Pediatrics, Division of Hematology-Oncology, University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School, New Brunswick 08903-0019, USA.
PURPOSE: Primary cardiac leiomyosarcoma is a rare malignant tumor in childhood. Patients with unresectable or partially resected cardiac leiomyosarcoma typically have a poor prognosis. The role of chemotherapy in the treatment of these patients has not been well defined. PATIENT AND METHODS: A 6-week-old infant with an incompletely resected cardiac leiomyosarcoma was treated postoperatively with ifosfamide and etoposide. RESULTS: The patient is disease-free and without apparent late effects 5 years following the completion of therapy. CONCLUSION: The combination of ifosfamide and etoposide warrants further evaluation in patients with leiomyosarcoma.
Ann Cardiol Angeiol (Paris) 1998 Jul-Sep;47(7):451-8
Leiomyosarcoma of the heart and great vessels
Babatasi G, Massetti M, Agostini D, Galateau F, Le Page O, Saloux E, Bhoyroo S, Grollier G, Potier JC, Khayat A.
Departement d'Anatomie Pathologique, CHU Caen Cote de Nacre, France.
Leiomyosarcomas (LMS) of the heart are exceptional primary malignant tumours with a catastrophic prognosis and a mean survival measured in months. Extensive radical surgical resection clearly remains the most appropriate treatment. We report three cases observed over a 3-year period, consisting of an LMS of the inferior vena cava, an LMS of the pulmonary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous vein graft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifurcation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart transplantation for local recurrence at the sixth month. The survival was significantly improved compared to other treatment options (chemotherapy, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedure and the medium-term follow-up of the transplanted patient revealed cerebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignant tumours. Recent progress of diagnostic investigations, such as spiral CT with reconstruction, MRI, positron emission tomography (PET), are now able to establish the diagnosis more rapidly and therefore allow more radical surgical resection. This resection, possibly combined with venous reconstruction, must be associated with adjuvant therapies. Heart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognosis of these lesions affections a young population.
Eur Radiol 1998;8(1):97-9
A recurrent left atrium leiomyosarcoma.
Durand E, Vanel D, Mousseaux E, Meingan P, Fornes P, Bittoun J.
Centre Inter-Etablissement de Resonance Magnetique, 78, rue du General Leclerc, F-94 275 Le Kremlin Bicetre, France.
Cardiac leiomyosarcoma is a rare tumour which can grow either intramurally or extramurally. Its prognosis is dismal. Surgery lengthens survival, but recurrences usually occur in the short term. Gated MRI is useful for the assessment of cardiac masses since it provides fine spatial and contrast resolution. We present the case of a left atrial leiomyosarcoma whose recurrence was diagnosed by gated MRI. Our patient underwent a second resection but died soon after.
Ann Cardiol Angeiol (Paris) 1998 Jul-Sep;47(7):451-8
Leiomyosarcoma of the heart and great vessels
Babatasi G, Massetti M, Agostini D, Galateau F, Le Page O, Saloux E, Bhoyroo S, Grollier G, Potier JC, Khayat A.
Departement d'Anatomie Pathologique, CHU Caen Cote de Nacre, France.
Leiomyosarcomas (LMS) of the heart are exceptional primary malignant tumours with a catastrophic prognosis and a mean survival measured in months. Extensive radical surgical resection clearly remains the most appropriate treatment. We report three cases observed over a 3-year period, consisting of an LMS of the inferior vena cava, an LMS of the pulmonary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous vein graft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifurcation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart transplantation for local recurrence at the sixth month. The survical was significantly improved compared to other treatment options (chemotherapy, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedure and the medium-term follow-up of the transplanted patient revealed cerebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignant tumours. Recent progress of diagnostic investigations, such as spiral CT with reconstruction, MRI, positron emission tomography (PET), are now able to establish the diagnosis more rapidly and therefore allow more radical surgical resection. This resection, possibly combined with venous reconstruction, must be associated with adjuvant therapies. Heart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognosis of these lesions affections a young population.
Arch Mal Coeur Vaiss 1994 Feb;87(2):291-4
Surgical treatment of leiomyosarcoma of the left atrium Report of a case and review of the literature
Boey S, Tribouilloy C, Lesbre JP, Stankowiak C, Copin MC, Haffreingue E, Espriet G.
Service de cardiologie, Clinique du Parc, Croix.
The authors report a case of left atrial leiomyosarcoma presenting with systemic disturbances and retinal emboli diagnosed by echocardiography and surgery in a 59 year old woman. Surgical ablation was completed by a course of chemotherapy. Histopathological examination confirmed the diagnosis of sarcoma; although the patient remained generally well, severe mitral regurgitation appeared 21 months after surgery. Transoesophageal echocardiography revealed an abnormal, hyper-mobile, intra-atrial echo suggesting a ruptured chordae tendinae and the mitral valves appeared very thickened and retracted. The patient was reoperated and the mitral valve replaced with a bioprosthesis. After a total follow-up of 29 months, the patients is still alive and asymptomatic. The authors underline the importance of echocardiography in the diagnosis of intra-cardiac tumours in general and, in particular, of intra-cardiac sarcomas.
Int J Cardiol 1997 Jun 27;60(1):91-4
Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy.
Pessotto R, Silvestre G, Luciani GB, Anselmi M, Pasini F, Santini F, Mazzucco A.
Division of Cardiac Surgery, OCM B.go Trento, Verona, Italy.
Primary cardiac sarcomas constitute a rare entity that have been uniformly associated with poor long-term survival. A case of left atrial leiomyosarcoma involving the interatrial septum and the right atrial free wall and presenting with syncope and atrial fibrillation, is described. Two extensive surgical excisions followed by adjuvant radiation and chemotherapy improved survival with a good quality of life. This approach of combined surgical, medical and radiation therapy may offer better longterm outcome, since our patient is the longest survivor thus far reported.
Ann Thorac Surg 2000 Oct;70(4):1412-4
Complete resection of cardiac leiomyosarcoma extending into the pulmonary trunk and right pulmonary artery.
Kono T, Takemura T, Hagino I, Matsumura G.
Department of Cardiovascular Surgery, National Nagano Hospital, Ueda, Japan. tetsuya@hsp.md.shinshu-u.ac.jp
A cardiac leimyosarcoma is an extremely rare tumor. We report a case of complete resection of a cardiac leiomyosarcoma extending into the pulmonary trunk and the right pulmonary artery using the Freestyle bioprosthesis (Medtronic, Inc, Minneapolis, MN) and Xenomedica graft (Baxter Healthcare Corp, Horw, Switzerland). Extensive resection and reconstructive surgery with the addition of radiotherapy prolonged the patient's life.
J Cardiovasc Surg (Torino) 2001 Feb;42(1):53-6
Primary cardiac leiomyosarcoma originating from the pulmonary valve. Case report and review of the literature.
Schroder S, Walker T, Greschniok A, Herdeg C, Karsch KR, Ziemer G.
Department of Internal Medicine, Division of Cardiology, University of Tubingen, Tubingen, Germany. Dr.Schroeder@t-online.de
Primary cardiac tumours are rare findings (incidence 0.02% according to a recent meta-analysis) with dismal prognosis. Approximately 25% are malignant, mostly represented by sarcomas. Among these, leiomyosarcomas are exceptional. Treatment for primary cardiac leiomyosarcomas consists of radical surgical resection followed by adjuvant radiation therapy and/or chemotherapy. The mean survival after surgery and adjuvant therapies is 6.8 months. We present a rare case of a 40- year-old male patient with a primary cardiac leiomysarcoma originating from the pulmonary valve. This patient died after surgery and implantation of a homograft of the pulmonary trunk. Furthermore, the literature has been reviewed.
Ann Thorac Surg 1991 Jun;51(6):999-1001
Primary cardiac leiomyosarcomas.
Antunes MJ, Vanderdonck KM, Andrade CM, Rebelo LS.
Department of Cardiothoracic Surgery, University of Coimbra, Portugal.
Two patients with primary cardiac leiomyosarcoma, one of the rarest malignant tumors of the heart, are described. The first patient had a tumor of the pulmonary trunk and was admitted with symptoms suggestive of pulmonary thromboembolism. The second had a tumor of the left atrium and had signs and symptoms of mitral valve obstruction and regurgitation. The 2 patients were operated on and later underwent adjuvant chemotherapy. Both died more than 22 months after operation, having required multiple hospital admissions for treatment of complications related to the pathology or the treatment of the primary cardiac leiomyosarcoma. Nonetheless, aggressive surgical excision followed by chemotherapy appears to have improved survival in these patients with primary cardiac leiomyosarcoma, as they are among the longest survivors reported.
Chin Med J (Taipei) 1997;59:136-40.
Leiomyosarcoma of the Left Atrium: A Case Report
Chin-Sung Kuo, Hui-Chi Hsu, Cheng-Hsiung Huang, Shian-Min Liu, Chao-Hung Ho
Primary malignant cardiac tumors are uncommon, and cardiac leiomyosarcoma is extremely rare. We reported a case of left atrial (LA) leiomyosarcoma with unusual clinical manifestations. A 28-year-old female presented with unknown cause of fever, body weight loss and anemia for two months. Echocardiography and magnetic resonance image study disclosed a 5x3x3.6 cm3 lobulated mass in the LA with invasion to its posterior wall. Histologic and immuno-histochemical studies of the resected specimen revealed a picture of leiomyosarcoma. The patient improved after surgical resection and post-operative chemotherapy. The literature was reviewed with a discussion of the clinical manifestations, diagnosis and treatment strategy of this rare tumor. Diagnosis of LA leiomyosarcoma is frequently delayed to make a very poor prognosis. Postoperative chemotherapy should be considered because of highly possible incomplete resection. However, an optimal treatment regimen remains unknown.
Thorac Cardiovasc Surg 1986 Dec;34(6):391-4
Surgery in primary leiomyosarcoma of the heart.
Segesser L, Cox J, Gross J, Lerch R, Gabathuler J, Glassey F, Gisselbaek A, Faidutti B.
Primary leiomyosarcoma of the heart is uncommon and we were able to find only 15 cases reported in the literature. Few of these cases were discovered during life and, as a result, even fewer have been treated surgically. We report a case of a right atrial leiomyosarcoma which was proven pathologically (histologically, immunohistochemically and ultrastructurally) after surgical resection. The clinical presentation, morphological, appearances and outcome are compared with those reported in the literature.
Surgery 1981 May;89(5):604-11
Resection of leiomyosarcoma originating in internal iliac vein and extending into heart via inferior vena cava.
Kaku K, Kawashima Y, Kitamura S, Nakano S, Mori T, Beppu SU, Kozuka T, Sakurai M, Katayama S, Tanizawa O, Sonoda T.
A patient's condition was initially diagnosed as constrictive pericarditis, but an echocardiogram revealed the presence of an intracardiac tumor. The tumor was a slow-growing leiomyosarcoma that originated in the left internal iliac vein, extended into the inferior vena cava, and reached the right ventricle. This tumor was considered to be a recurrent uterine leiomyosarcoma that had been resected 4 years before. After a second resection of this tumor, the patient was living a normal life 61/2 years postoperatively, in spite of recurrence in the pelvis and lung. We reviewed 12 cases of leiomyosarcoma in the world literature that reached the heart through the inferior vena cava. We added our case and evaluated the clinical features of all 13. This is the first successfully resected case of leiomyosarcoma of this nature so far as we could determine.
Back to E-chocardiography Home Page.
The contents and links on this page were last verified on October 31, 2006.