Interactive CardioVascular and Thoracic Surgery 19 (2014) 1077–1079 doi:10.1093/icvts/ivu298 Advance Access publication 8 September 2014

Extraskeletal chondroblastic osteosarcoma in the left atrium Özge Korkmaza,*, Sabahattin Göksela, Hatice Reyhan Eg˘ ilmezb and Öcal Berkana a b

Department of Cardiovascular Surgery, Cumhuriyet University, Sivas, Turkey Department of Pathology, Cumhuriyet University, Sivas, Turkey

* Corresponding author. Department of Cardiovascular Surgery, Cumhuriyet University, 58140 Sivas, Turkey. Tel: +90-346-2581905; e-mail: [email protected] (Ö. Korkmaz). Received 23 April 2014; received in revised form 23 July 2014; accepted 1 August 2014

Abstract Primary cardiac extrasceletal osteosarcomas are uncommon tumours. They have aggressive behaviour and thus poor prognosis. This report describes a 40-year old female patient who was referred to our hospital with dyspnoea, weakness and syncopal attacks. Echocardiography and chest computed tomography showed a left atrial mass, and tumour excision revealed a cardiac chondroblastic osteosarcoma. After pathological diagnosis, she was scheduled for chemotherapy and radiotherapy by the related departments.

INTRODUCTION Primary cardiac tumours are very rare with a prevalence rate ranging from 0.001 to 0.03% in autopsy series [1]. Most primary cardiac tumours are benign, such as myxoma, lipoma, papillary fibroelastoma and rhabdomyoma. Angiosarcomas and myxofibrosarcomas are the most common sarcomas of the heart. However, a primary cardiac chondroblastic osteosarcoma in the heart is a unique case, due to the fact that this type is considered to be a member of the extraskeletal osteosarcoma (ESOS) family. ESOS is an uncommon malignant neoplasm, which can produce osteoid, bone or chondroid matrix, located in the soft tissue without connection to the skeleton. The most common location is the lower extremity, especially the thigh, followed by the upper extremity and the retroperitoneum [2]. Herein, we describe the gross and histological findings of a 40-year-old woman who presented with an extraskeletal chondroblastic osteosarcoma (also of known chondroblastic subtype), originating from the heart and extending into the ostium of the right superior and inferior pulmonary veins. To the best of our knowledge, this the first report of a chondroblastic osteosarcoma arising in the heart.

CASE A 40-year old woman was referred to our clinic due to dyspnoea, weakness and syncopal attacks after physical activity. She had no history of lung cancer or radiotherapy or any other cancers. During her physical examination, accentuated S1 and a mild systolic murmur without diastolic rumble were noted. On her chest X-ray, a double-density sign due to left atrial enlargement was noted. Her blood counts and laboratory tests were within normal limits. Transthoracic echocardiography revealed a left atrial mass measuring 3.5 × 3 cm in diameter, connecting to the interatrial

septum, filling the left atrial cavity and extending into the right inferior pulmonary vein. Transoesophageal echocardiography (TEE) revealed that this mass was continuous with the posterior wall of the left atrium with a diameter of 4.5×5 cm. The core of the mass had calcific parts. A thoracoabdominopelvic computed tomographic scanning study showed a mass with calcification in the central part, which filled the left atrial cavity (Figs. 1a and 1b). In light of all these findings, the presumptive diagnosis of cardiac myxoma was made and along with the symptoms, the decision was made to excise the tumour via the left and right atrial approach, as it extended to both the superior and inferior right pulmonary veins and was connected to the atrial septum. During the operation, the interatrial septum attached to the segment was removed with the mass. After the operation, the gross section of the specimen showed an 8 × 4 × 4 cm solid tumoural mass, which was covered by a fibrous sheet. When the tumoural mass was cut with a surgical blade, it was shown that semisoft connective tissue covered the calcified core. When the left atrium was closed, TEE was performed. According to both intraoperative assessment and TEE, mitral valve functions were within normal ranges. The surgically resected specimen was found to have a distinct pseudocapsule. It was firm to stony on palpation. On section, it displayed a granular white surface with yellow flecks and haemorrhage. Microscopically, the presence of neoplastic osteoid, bone and cartilage of variable cellularity with bone formation was noted (Figs. 2a and 2b). There were fibroblastic elements and atypical cartilage of variable cellularity with bone formation. No necrosis was observed. Following the operation, the patient was followed up for 2 days in the cardiovascular intensive care unit. Owing to the possibility of a further primary tumour or distant metastasis, a bone scan was performed; the bone scan was normal, without any sign of distant metastasis or any other primary tumour. All these findings established the final diagnosis that the tumour originated from the heart.

© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.


Keywords: Chondroblastic osteosarcoma • Heart • Extraskeletal • Primary


Ö. Korkmaz et al. / Interactive CardioVascular and Thoracic Surgery

Figure 1: (A) Computerized tomographic chest scan showed that mass filling the left atrial cavity. (B) The surgically resected specimen.

Figure 2: (A, B) Extraskeletal chondroblastic osteosarcoma with fusiform cells separated by osteoid and neoplastic cartilage (H & E, ×25). H & E: Haematoxylin and eosin.

The pathological examination revealed chondroblastic osteosarcoma arising from the left atrial wall and the interatrial septum. The patient was discharged on the 10th day postoperatively, after she had consulted the medical oncology department for chemotherapy and the radiation oncology department for her radiotherapy schedule.

DISCUSSION ESOS, which is a malignant mesenchymal neoplasm, produces osteoid, bone or chondroid material, and it is commonly located in soft tissues without attachment to the skeleton. It is rare, and is reported in fewer than 2% of all soft tissue sarcomas [2]. The heart is an extraordinary site for osteosarcoma. In the current case, both clinical and radiographic evaluations failed to reveal any tumours at other sites. The pathogenesis of the tumour was not clear, but in the metaplasia of connective tissue, it suggests a malignant degeneration of embryonic somatic residues [3]. The proposed aetiological factors are mechanical injury by causative agents and the following radiotherapy [4]. With regard to primary involvement of the heart, these hypotheses are vague. However, the heart is also known to have a skeleton, namely, fibrous trigone. Mitral, aortic and pulmonary annuli make up the left trigone. The annulus is the essential part of the trigone. Histologically, the annulus has fibrotic and elastic fibres

and this structure is also in continuity with the leaflet tissue, the atrium and the ventricles. The researchers’ hypothesis of the pathophysiology of ESOS in the heart is based on malignant metaplasia and transformation of fibrotic and elastic fibres, which make up the trigone. The patient with ESOS could have been asymptomatic, but symptoms can occur due to an enlarging tumoural mass. The patient in the current study suffered from dyspnoea, weakness and syncopal attacks after physical activity due to left atrial obstruction by a tumour, and this prevented left ventricular filling. The treatment of choice is surgical resection, so that mechanical obstruction can be eliminated. Some cases might be treated by radiotherapy or systemic chemotherapy although these treatments are not well established. ESOS prognosis is poor with a 5-year survival rate of less than 25% [5]. However, the patient in the current study is still under treatment in the medical oncology department.

CONCLUSION The current study reported a primary cardiac chondroblastic osteosarcoma, which is a subtype of ESOS and in which the heart is an uncommon localization. Unfortunately, this tumour has aggressive behaviour and poor prognosis. Conflict of interest: none declared.

Ö. Korkmaz et al. / Interactive CardioVascular and Thoracic Surgery

[1] Burke A, Virmani R. Tumors of the heart and great vessels. In: Atlas of Tumor Pathology, Third Series, Fascicle 16. Washington DC: Armed Forces Institute of Pathology, 1996: 21–46. [2] Klein MJ, Siegal GP. Osteosarcoma: anatomic and histologic variants. Am J Clin Pathol 2006;125:555–81.

[3] Strak P, Smith DC, Watkins GE, Chun KE. Primary intrathoracic extraosseous osteogenic sarcoma: report of three cases. Radiology 1990;174:725–26. [4] Sordillo PP, Hajdu SI, Magilla GB, Golbey RB. Extraosseous ostoegenic sarcoma. A review of 48 patients. Cancer 1983;51:727. [5] Lidang Jensen M, Schumacher B, Myhre Jensen O, Steen Nielsen O, Keller J. Extraskeletal osteosarcomas: a clinicopathologic study of 25 cases. Am J Surg Pathol 1998;22:588–94.




Extraskeletal chondroblastic osteosarcoma in the left atrium.

Primary cardiac extrasceletal osteosarcomas are uncommon tumours. They have aggressive behaviour and thus poor prognosis. This report describes a 40-y...
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