Asian Cardiovasc Thorac Ann 2001;9:68-70
© 2001 Asia Publishing EXchange Pte Ltd
Heart Leiomyosarcoma Mimicking Pulmonary Thromboembolism
Hu Sheng Shou, MD,
Sun Cheng Chao, MD,1,
Zheng Zhe, MD
Department of Cardiovascular Surgery Fuwai Hospital, Peking Union Medical College Beijing, People's Republic of China
1 Department of Cardiac and Thoracic Surgery First Affiliated Hospital, Wenzhou Medical College Wenzhou, Zhejiang Province People's Republic of China
|
|
|
For reprint information contact: Hu Sheng Shou, MD Tel: 86 10 6831 4466 Ext. 8359 Fax: 86 10 6831 3012 email: zhengzhe{at}263.net Department of Cardiovascular Surgery, Fuwai Hospital, Peking Union Medical College, 167 Beilishi Road, Beijing 100037, People's Republic of China.
|
 |
Abstract
|
|---|
A 34-year-old man was admitted with a putative diagnosis of pulmonary artery trunk thromboembolism. Magnetic resonance imaging and angiography showed a solid mass extending from the pulmonary trunk to the right pulmonary artery. During surgery, a tumor was found arising from the pulmonary valve annulus. The tumor was completely removed and pulmonary valvuloplasty was performed. The result was satisfactory. Histopathology showed the tumor to be a leiomyosarcoma.
|
Introduction
|
|---|
Leiomyosarcoma rarely occurs in the heart and a sarcoma arising from the pulmonary valve ring is extremely uncommon. A tumor located in the pulmonary valve region may be suspected to be a pulmonary thromboembolism and it would cause apparent pulmonary valve stenosis.
|
Case Report
|
|---|
A 34-year-old man was admitted with a 7-month history of progressive exertional dyspnea and an episode of syncope. There was a soft grade 3/6 systolic murmur at the second to fourth intercostal spaces on auscultation. Echocardiography showed an echodense mass in the pulmonary valve and supravalvular area. The right atrium and right ventricle were enlarged and there was moderate-to-severe tricuspid regurgitation. Magnetic resonance imaging revealed a solid mass extending from the pulmonary trunk to the right pulmonary artery, and the pulmonary trunk was enlarged. Digital subtraction angiography demonstrated a filling defect above the pulmonary valve and absence of filling of the right main pulmonary artery (Figure 1
). The patient underwent surgery with a putative diagnosis of pulmonary thrombo-embolism.
View larger version (161K):
[in this window]
[in a new window]
|
Figure 1. Preoperative digital subtraction angiogram revealing a filling defect above the pulmonary valve and absence of filling of the right main pulmonary artery.
|
|
A median sternotomy was performed and standard cardiopulmonary bypass was established via bicaval venous drainage and aortic return. When the pulmonary artery trunk was incised, a tumor was revealed. The pedicle was located on the valve annulus at the commissure of the right and left pulmonary cusps, and one half of the left cusp rode above it. The tumor did not infiltrate the surrounding structures but extended intraluminally from the pulmonary trunk to the right main pulmonary artery and the middle pulmonary artery. The pulmonary trunk was almost occluded and the right main pulmonary artery was completely blocked. The half of the left pulmonary cusp on the tumor pedicle was resected first. The tumor pedicle was completely removed with partial resection of the pulmonary annulus. Continuous sutures of 4/0 polypropylene were used to reinstate the free part of the left cusp to its anatomic position, and an annuloplasty suture was placed at the commissure of the right and left pulmonary cusps to restore valve function. The excised mass measured 2 cm in diameter and 5 cm in length. Histopathological and immunohistochemical examination demonstrated that it was a high-grade differentiated leiomyosarcoma. Postoperative digital subtraction angiography showed normal filling of the pulmonary artery trunk (Figure 2). The patient was discharged without additional antineoplasm therapy and he was still alive with no echocardiographic evidence of recurrence 10 months after the operation.
View larger version (173K):
[in this window]
[in a new window]
|
Figure 2. Postoperative digital subtraction angiogram showing normal filling of the pulmonary artery trunk and its two main branches.
|
|
|
Discussion
|
|---|
Primary cardiac leiomyosarcoma is very uncommon. Sutsch and colleagues1 found only one case in 20,305 consecutive patients undergoing echocardiographic studies. Basso and colleagues2 reported 2 leiomyosar-comas in a review of 125 biopsy-documented primary cardiac and pericardial tumors. A leiomyosarcoma originating from the pulmonary valve is extremely rare.
The symptoms of primary cardiac leiomyosarcoma are nonspecific. If the tumor mass is located in the right ventricular outflow track, pulmonary valve, or pulmonary artery trunk, it will invariably mimic pulmonary thrombo-embolism and thus be misdiagnosed.3–5 Zerkowski and colleagues5 estimated that 70% of patients in reported cases were operated upon with a presumed diagnosis of thromboembolism. Transesophageal color Doppler echocardiography or magnetic resonance imaging with gadolinium diethylene triaminepentaacetic acid may be useful for differentiation.6
The principle in treating a cardiac sarcoma is removal of the tumor as completely as possible. After radical resection of a tumor in the pulmonary valve region, reconstruction is usually needed with either a patch, prosthesis, or homograft. Mazzucco and colleagues7 reported a similar case of cardiac leiomyosarcoma in 1994, in which the tumor arose from the posterior leaflet of the pulmonary valve and was firmly attached to the myocardium of the infundibular septum. Transvalvular cuneiform myectomy of the infundibular septum and resection of the posterior pulmonary valve leaflet were performed to completely ablate the tumor; the patient did well without evidence of recurrence in 8 months of follow-up. Zerkowski and colleagues5 reported a case of hemangiosarcoma origi-nating from the pulmonary valve; the pulmonary trunk was replaced with a homograft.
The prognosis of primary cardiac leiomyosarcoma is poor and mean survival after diagnosis is 6 months.3 Krüger and colleagues8 analyzed 93 cases of primary pulmonary artery sarcoma and found that survival was less than 1.5 months without treatment, but extended to 10 months after surgical resection. Whether cases of cardiac sarcoma should receive adjuvant chemotherapy or radiotherapy after surgical resection is controversial.5 Because cardiac sarcomas are so uncommon, experience of treatment is limited. Our patient's tumor was completely resected and the pathological diagnosis was high-grade differentiated leiomyosarcoma, so no further therapy was adopted. Timely diagnosis and radical surgical excision may have prolonged his survival. Primary cardiac sarcomas should be considered in differential diagnosis of pulmonary embolism.
|
References
|
|---|
-
Sutsch G, Jenni R, von Segesser L, Schneider J. Heart tumors: incidence, distribution, diagnosis. Exemplified by 20,305 echocardiographies [German]. Schweiz Med Wochenschr 1991;121:621–9.[Medline]
-
Basso C, Valente M, Poletti A, Casarotto D, Thiene G. Surgical pathology of primary cardiac and pericardial tumors. Eur J Cardio-thorac Surg 1997;12:730–7.[Abstract]
-
Antunes MJ, Vanderdonck KM, Andrade CM, Rebelo LS. Primary cardiac leiomyosarcomas. Ann Thorac Surg 1991; 51:999–1001.[Abstract]
-
Shimono T, Yuasa H, Yuasa U, Yasuda F, Adachi K, Tokui T, et al. Pulmonary leiomyosarcoma extending into left atrium or pulmonary trunk: complete resection with cardiopulmonary bypass. J Thorac Cardiovasc Surg 1998; 115:460–1.[Free Full Text]
-
Zerkowski HR, Hofmann HS, Gybels I, Knolle J. Primary sarcoma of pulmonary artery and valve: multimodality treatment by chemotherapy and homograft replacement. J Thorac Cardiovasc Surg 1996;112:1122–4.[Free Full Text]
-
Suzuki M, Hamada M, Abe M, Matsuoka H, Shigematsu Y, Sumimoto T, et al. Accurate diagnosis of metastatic cardiac leiomyosarcoma with infundibular stenosis and cardiac tamponade by transesophageal echocardiography and Gd-DTPA magnetic resonance imaging — report of a case. Jpn Circ J 1994;58:222–6.[Medline]
-
Mazzucco A, Luciani GB, Bertolini P, Faggian G, Morando G, Ghimenton C. Primary leiomyosarcoma of the pulmonary artery: diagnostic and surgical implications. Ann Thorac Surg 1994;57:222–5.[Abstract]
-
Krüger I, Borowski A, Horst M, de Vivie ER, Theissen P, Gross-Fengels W. Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Surg 1990;38:91–5.[Medline]
TOP
Abstract
Introduction
