Indian Journal of Pathology and Microbiology
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Year : 2008  |  Volume : 51  |  Issue : 1  |  Page : 30-31
Primary cardiac sarcoma

1 Department of Pathology, Kasturba Medical College, Mangalore, Karnataka, India
2 Department of Cardiothoracic Surgery, AJ Hospital and Research Institute, Mangalore, Karnataka, India
3 Department of Anaesthesiology, AJ Hospital and Research Institute, Mangalore, Karnataka, India

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Primary cardiac sarcomas are uncommon entities. The diagnosis of sarcoma is not made preoperatively because of the rarity of the lesion and the nonspecific nature of the signs and symptoms. This is a report of a case of primary sarcoma arising from the left atrium, in a patient who presented with acute dyspnoea; the sarcoma resembled an atrial myxoma on echocardiography.

Keywords: Malignant fibrous histiocytoma, primary cardiac sarcoma

How to cite this article:
Khadilkar U N, Harish R, Tantry G, Kumar S, Tiwari A. Primary cardiac sarcoma. Indian J Pathol Microbiol 2008;51:30-1

How to cite this URL:
Khadilkar U N, Harish R, Tantry G, Kumar S, Tiwari A. Primary cardiac sarcoma. Indian J Pathol Microbiol [serial online] 2008 [cited 2023 Jun 6];51:30-1. Available from:

   Introduction Top

Primary cardiac sarcomas are rare with an autopsy prevalence of 0.001-0.28%.The common malignant tumor of the heart and pericardium is sarcoma comprising about 25% of primary cardiac tumors. [1] In decreasing order of frequency, the sites involved are the right atrium, left atrium, right ventricle, and left ventricle. The most frequent sarcoma is angiosarcoma, which most often originates in the right atrium. [2] The other sarcomas are rhabdomyosarcoma, fibrosarcoma, liposarcoma, lymphoma, myxosarcoma, and malignant histiocytoma. Echocardiography, especially transoesophageal echocardiography is very effective for demonstrating the intracardiac components of the sarcoma and its effect on cardiac valves. Magnetic resonance imaging (MRI) is the most effective technique for staging cardiac sarcomas by demonstrating both the intracardiac and extracardiac components.

   Case History Top

A 31-year-old lady presented with an acute-onset of breathlessness to the cardiothoracic department; echocardiography showed a mass attached to the left posterior atrial wall and extending into the left ventricle, and a diagnosis of atrial myxoma was considered. Peroperatively, the tumor measured 4 x 3 cm 2 , attached by a broad base to the posterior atrial wall and to the posterior mitral leaflet and also extended into the pulmonary vein. The tumor was removed piece-meal, as complete removal was not possible due to the extension into the adjacent structures, and sent for histopathologic examination.

Pathological findings

The tumor tissue comprised of multiple grey-white to yellowish bits, the largest of which measured 4 x 2.5 x 1 cm 3 ; some of the bits were covered by a smooth lining [Figure - 1].

Microscopically the paraffin sections of the tumor tissue showed, a pleomorphic malignant neoplasm composed of long and short fascicles of spindle-shaped cells with eosinophilic cytoplasm, elongated nuclei with pointed ends, prominent nucleoli, and rounded malignant cells with enlarged nuclei and prominent nucleoli. Many giant cells were interspersed with the tumor cells and the mitoses were prominent. Foci of tumor necrosis were also seen [Figure - 2].

Special histochemical stains viz ., van-Geison's, Masson's trichrome, and phosphotungstic acid, confirmed the presence of fibroblastic, and absence of smooth muscle and rhabdomyoblastic differentiation in the tumor cells. Immunohistochemistry showed positivity with vimentin [Figure - 3] and nonreactivity with desmin and smooth muscle actin. The tumor conformed to a histopathologic diagnosis of malignant fibrous histiocytoma (MFH) based on the morphology and histochemistry.

   Discussion Top

The left atrium is the predominant site for connective tissue sarcomas, which protrude into the cavity and thus on echocardiography, can be easily mistaken for atrial myxomas. Rapid growth and embolic phenomenon are common to all atrial sarcomas. [3]

Sarcomas are usually regarded as arising from undifferentiated mesenchymal cells in the endocardium. In practice most sarcomas consist of spindle-shaped cells with a high mitotic rate and show no particular differentiation pattern. For all these reasons, it is difficult to gain a clear impression of the prognosis of cardiac sarcomas in relation to tumor type. [3] Better survival has been shown to be associated with the absence of necrosis, low mitotic count, and left-sided situation. The last attribute, reflects early diagnosis rather than inherently different behavior. [3]

Malignant fibrous histiocytoma generally appears as a whitish-grey, multilobulated invasive mass. Prognosis of MFH is poor, but the occasional case of survival for 18 months after incomplete surgical excision has been reported. [3]

In the present study, the tumor mimicked an atrial myxoma on echocardiography as more than 90% of atrial myxomas are located in the left atrium, are slightly more common in women than in men, and are soft masses, sessile, or pedunculated, with a pedicle attached to the limbus of the fossa ovalis. The possibility of the tumor mass being a sarcoma was considered because of the origin from the posterior atrial wall, extension into adjacent structures, and the difficulty encountered in separating the tumor from the underlying atrial myocardium at surgery. The histologic appearance resembled MFH and the fibroblastic nature was confirmed with histochemistry and immunoreactivity with vimentin. An early diagnosis was possible because of the left-sided location, but the tumor manifested an unfavorable histology with increased mitoses and foci of necrosis. An echocardiography done 1 month after surgery did not reveal any residual tumor. And the patient is being followed up regularly to date, 6 months after surgery.

Osteosarcoma arising from the left atrium is usually confused with obstructive myxoma and presents as a surgical surprise. [4] Echocardiography also cannot differentiate it from myxoma, but for the larger size, areas of calcification and extension into the pulmonary veins or mitral valve. Preoperative suspicion of malignant cardiac neoplasm can be extremely useful for the surgeon, to prepare for an extensive resection. [5]

Primary cardiac sarcomas present an unusual and difficult surgical challenge. Malignant tumors of the left atrium have proved problematic due to their posterior location and difficulty of surgical exposure. The technique of cardiac explantation, ex vivo resection, and cardiac reconstruction, and reimplantation - the cardiac autotransplantation procedure has been developed to solve this anatomic problem. [6] As of now the recommended treatment option for cardiac sarcomas is wide surgical excision with adjunct chemotherapy. [7]

   References Top

1.Burke AP, Cowan D, Virmani R. Primary cardiac sarcoma of the heart. Cancer 1992;69:387-95.  Back to cited text no. 1  [PUBMED]  
2.Amonkar GP, Deshpande JR. Cardiac angiosarcoma. Cardiovasc Pathol 2006 Jan-Feb;15:57-8.  Back to cited text no. 2    
3.Davies MJ, Mann JM. Acquired diseases of the heart. Cardiac tumors. 3 rd ed. New York: Churchill Livingstone; 1995. p. 288-90.  Back to cited text no. 3    
4.Putnam JB, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg 1991;51:906-10.  Back to cited text no. 4    
5.Mathur A, Yadav RS, Sharma A, Khanna H, Arsiwala S. Osteosarcoma of the left atrium. IJTCVS 2005;21:279-80.  Back to cited text no. 5    
6.Conklin LD, Reardon MJ. Auto-transplantation of the heart for primary cardiac malignancy- Development and surgical technique. Tex Heart Inst J 2002;29:105-8.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Shanmugan G. Primary cardiac sarcoma. Eur J Cardiothoracic Surgery 2006 Jun;29:925-32.  Back to cited text no. 7    

Correspondence Address:
U N Khadilkar
Department of Pathology, Kasturba Medical College, P. O. Box No. 53, Mangalore 575 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.40387

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  [Figure - 1], [Figure - 2], [Figure - 3]


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