Cancer Res Treat.  2007 Sep;39(3):131-133.

Extraskeletal Mesenchymal Chondrosarcoma of the Heart Responded to Systemic Chemotherapy: A Case Report

• Hsing CT
• Oh SY
• Lee S
• Kwon HC
• Kim SH
• Park TH
• Woo JS
• Na SH
• Kim HJ

Abstract

Mesenchymal chondrosarcoma is a rare cartilaginous neoplasm of an extraskeletal origin, and this predominately occurs in the head and neck, and also in the lower extremities. Fewer than twenty cases of cardiac mesenchymal chondrosarcoma have so far been reported on. For the most part, the results of treatment for patients with this condition have been dismal. In this study, we describe a case of cardiac mesenchymal chondrosarcoma that responded to chemotherapy following surgical biopsy. A 46-year-old man was referred for evaluation of his pleural effusions in both lungs. Chest computed tomography revealed an ovoid-shaped mass in the posterior wall of the patient's left atrium. The echocardiogram revealed a large ovoid-shaped immobile mass (11 x 6 cm2) in the pericardiac space, which was attached to the posterior wall of the left atrium. Emergency pericardiostomy with closure thoracostomy was performed. Seven days later, a thoracotomy was performed for reduction and diagnosis of the cardiac mass. The pathological diagnosis was extraskeletal mesenchymal chondrosarcoma of the heart.. Postoperative chemotherapy was performed for the huge remaining mass with a combined regimen of etoposide, ifosfamide and cisplatin. After 6 cycles, the patient showed a partial response without symptoms. Although cardiac mesenchymal chondrosarcoma has been reported to be chemotherapy- resistant with a short survival duration, chemotherapy may prove to be an effective treatment modality.

Keyword

Mesenchymal chondrosarcoma; Extraskeletal; Heart

MeSH Terms

Biopsy
Chondrosarcoma, Mesenchymal*
Cisplatin
Diagnosis
Drug Therapy*
Emergencies
Etoposide
Head
Heart Atria
Heart*
Humans
Ifosfamide
Lower Extremity
Lung
Middle Aged
Neck
Pericardial Window Techniques
Pleural Effusion
Thoracostomy
Thoracotomy
Thorax
Cisplatin
Etoposide
Ifosfamide

Figure

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  Fig. 1 Chest computed tomography (A) and echocardiogram (B) revealed a large ovoid immobile mass (11×6 cm2) in the pericardial space, which was attached to the posterior wall of the LA, and this was accompanied by a large quantity of pericardial effusion. Chemotherapy for six cycles was followed by chest computed topography (C) and an echocardiogram (D). The size of the immobile mass attached to the posterior wall of the LA was reduced (from 4.4×3.8 cm2 to 1.8×2.5 cm2).

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  Fig. 2 Pathologic findings of the extraskeletal mesenchymal chondrosarcoma. (A) H&E stain (B) S-100 (×400) and (C) CD99 stain (×400).

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  Fig. 3 PET CT showed no uptake in the cardiac mass lesion and no evidence of distant metastasis.

Reference

1. Izzo P, Ricci N, Capolupo R, Villani PC, Federici F, Roma F, Curcio C. A rare care of primary chondrosarcoma of the heart. J Cardiovasc Med. 2007; 8:210–213.

2. Miwa S, Konishi Y, Matsumoto M, Minakata K. Primary cardiac chondrosarcoma: a case report. Jpn Circ J. 1997; 61:795–797. PMID: 9293411.

3. Kaneko T, Suzuki Y, Takata R, Takata K, Sakuma T, Fujioka T. Extraskeltal mesenchymal chondrosarcoma of the kidney. Int J Urol. 2006; 13:285–286. PMID: 16643625.

4. Ng SH, Ko SF, Yang TS, Wong HF, Wan YL, Ho YS. Primary cardiac chondrosarcoma. Am J Emerg Med. 1996; 14:285–287. PMID: 8639204.

5. Nesi G, Pedemonte E, Gori F. Extraskeletal mesenchymal chondrosarcoma involving the heart: report of a case. Ital Heart J. 2000; 1:435–437. PMID: 10929746.