|Year : 2019 | Volume
| Issue : 1 | Page : 86-87
Primary cardiac osteosarcoma: A rare cause of chest pain in a young man
Rambod Mozafari1, Zahra Mohebbi2, Tina Shooshtarizadeh3, Hamed Sotoude4
1 Department of Hematology, Oncology, and Bone Marrow Transplantation, Dr Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
2 Department of Internal Medicine, Kurdistan University of Medical Sciences, Sanandaj, Iran
3 Department of Pathology, Iran University of Medical Sciences, Tehran, Iran
4 Department of Emergency Medicine, Kurdistan University of Medical Sciences, Sanandaj, Iran
|Date of Web Publication||4-Apr-2019|
Department of Emergency Medicine, Kurdistan University of Medical Sciences, Sanandaj
Source of Support: None, Conflict of Interest: None
Although primary cardiac osteosarcoma is uncommon, cardiac neoplasms should be in the differential diagnosis of cardiac complaints. We present a case of a 35 year old man with recurrent episodes of exertional chest pain and who was diagnosed with primary cardiac osteosarcoma. Cardiac osteosarcomas are rare but must be suspected.
Keywords: Cardiac neoplasm, chest pain, osteosarcoma
|How to cite this article:|
Mozafari R, Mohebbi Z, Shooshtarizadeh T, Sotoude H. Primary cardiac osteosarcoma: A rare cause of chest pain in a young man. Indian J Cancer 2019;56:86-7
| » Introduction|| |
Cardiac complaints are one of the most common reasons for seeking medical care. Coronary artery diseases, congestive heart failure, valvular diseases, and arrhythmias are the primary etiologic causes. Cardiac neoplasms may have similar presentations but have been often overlooked because of rare occurrence. In most instances cardiac tumors are metastases. Myxomas are the prototypical primary benign cardiac tumors, and sarcomas occupy the first place in primary cardiac malignancies. We present a case of primary cardiac osteosarcoma, which is a very rare subtype of cardiac sarcoma. This case teaches us that having knowledge about uncommon differentials and taking them into consideration in the appropriate context is an important, inseparable part of medicine to be learned and used in practice.
| » Case Report|| |
A healthy 35-year-old man presented to the emergency department with 1-month history of episodic exertional chest discomfort during exercise. He had the habit of weekly mountain climbing and running. On physical examination, there was nothing significant except a mild systolic murmur at the left sternal border. An electrocardiogram (ECG) showed normal sinus rhythm, and the laboratory results were within normal limits. He had several medical consultations after the onset of symptoms and was attributed a non-cardiac pathology based on ECG and an exercise tolerance test with no obvious abnormal finding. A prominent left atrium (LA) chest X-ray directed to echocardiographic examination which revealed a large (33 × 30 mm) round-shape heterogenous highly mobile mass in LA [Figure 1] adhering to anterior mitral valve leaflet and protruding into the left ventricle during diastole.
On diagnosing a cardiac tumor, probably atrial myxoma, the patient underwent surgical intervention. Macroscopically, the excised specimen contained two sections: an atrial mass measuring 3.8 × 3 × 2 cm (a multilocular hemorrhagic cystic lesion with a grayish color) and an atrial mass stalk (a grayish tissue) measuring 0.7 × 0.6 × 0.4 cm. Histopathologic examination revealed neoplastic growth of highly atypical and mitotic malignant mesenchymal cells (pleomorphic sarcoma) in a myxoid stroma with multiple cystic spaces containing blood and foci of osteoid and woven bone formation with large atypical osteoblasts (oval to-spindle shaped appearance) [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d, which was compatible with the diagnosis of cardiac osteosarcoma. Immunohistochemical study of tumor cells was positive for desmin in some cells and negative for CD31, CD34, SMA, S100, and calretinin. Postoperative follow-up including whole-body bone scan [Figure 3] and brain, chest, and abdominopelvic computed tomography (CT) showed no abnormal findings, and the was diagnosed as primary cardiac osteosarcoma, with no apparent metastasis.
|Figure 2: Pathologic panel showing pleomorphic sarcoma forming large trabecula of woven bone (a) and irregular sheets of osteoid (b), sheets of malignant oval to spindle cells with marked nuclear atypia and frequent mitotic figures (c) forming thin trabecula of osteoid (d)|
Click here to view
|Figure 3: Whole-body bone scan with no evidence of active lesion except in the sternum due to sternotomy|
Click here to view
After surgery, the patient was discharged in good general condition. He was on follow-up program and there was no recurrence until 9 months.
| » Discussion|| |
In general, cardiac neoplasms are among the most infrequent of tumors. They are classified into primary or secondary metastatic malignant tumors in the heart. Primary cardiac neoplasms are about 30 times less common than secondary neoplasms. These tumors have a prevalence rate of 0.1–0.3% based on most autopsy series., Primary cardiac tumors are often benign with atrial myxoma being the most common type and mostly located in LA., Malignant primary tumors constitute <25% of all primary cardiac neoplasms and majority of them are sarcomas, primarily angiosarcomas or undifferentiated sarcomas.,, Primary cardiac osteosarcoma, a subtype of cardiac sarcomas, is extremely rare. In contrast to metastatic ones, primary cardiac osteosarcomas involve predominantly LA,, and hence, are often been confused with atrial myxomas in radiological studies. Although obstructive effects and heart failure are the typical manifestations, it has various shapes and presentations.,
Currently, echocardiography cannot reliably differentiate an osteosarcoma and a myxoma, but certain characteristics such as large size, immobility of the mass, neovascularity, absence of a clear pedicle, multicentricity, calcification and invasion into the pulmonary veins, valvular structures, and atrial septum may suggest malignancy. Cardiac osteosarcomas can be seen as a low attenuation mass with dense calcification on CT but this is a neither sensitive nor specific finding and can been observed in other tumors such as myxomas, fibromas, rhabdomyomas, and teratomas too. Macroscopic appearance of the tumor may be similar in myxomas and sarcomas. Hence pathologic examination is essential to the diagnosis. Spindle cells, osteoid, bone, and cartilage in variable amounts are histologic features of the tumor.
The mainstay of treatment is surgery. Because of the low incidence of the tumor and sensitivity of cardiac tissue, the role of chemotherapy and radiotherapy is controversial. Cardiac osteosarcoma is an aggressive tumor with high metastasis and recurrence rates, and for this reason patients often have poor survival. Metastasis of distant organs including the skin, brain, thyroid, lung, stomach, liver, kidney, muscle, and bone has been reported.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that the name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| » References|| |
Roberts WC. Primary and secondary neoplasms of the heart. Am J Cardiol
Aguilar CA, Donet JA, Galarreta CI, Yabar A. A primary cardiac osteosarcoma: Case report and review of the literature. J Cardiol Cases
Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer
Bae JH, Hong G, Lee S, Kim W, Park J, Shin D, et al.
Primary cardiac osteosarcoma. Korean Circ J
Burke AP, Virmani R. Osteosarcomas of the heart. Am J Surg Pathol
Vander Salm TJ. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg
Luo H, Lei Y, Su C, Cai L, Wang T, Zou J, et al
. Primary cardiac osteosarcoma in a 42-year-old woman. J Cardiothorac Surg
Mich RJ, Gillam LD, Weyman AE. Osteogenic sarcomas mimicking left atrial myxomas: Clinical and two-dimensional echocardiographic features. J Am Coll Cardiol
[Figure 1], [Figure 2], [Figure 3]