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Primary pericardial malignant fibrosarcoma presenting as sudden onset of substernal pain
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  1. S-Y Yoo,
  2. C-B Park,
  3. S S Cheong
  1. sscheong{at}gnah.co.kr

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A malignant fibrosarcoma originating from pericardium is quite rare. A 30-year-old man was hospitalised for sudden-onset severe, substernal and pleuritic chest pain after strenuous exercise. Transthoracic echocardiography (panel A) showed a large amount of pericardial effusion and a multiple-echogenic mass (arrows) attached to the pericardium. Magnetic resonance coronal T2WI imaging (panel B) showed ovoid-shaped solid mass consisting of intermediate (small arrows) and low signal intensity (large arrow) in the right pericardial space. The patient underwent a complete resection of the tumour with a partial pericardiectomy. Operative findings showed a large amount of haemorrhagic fluid and a three-lobed mass (arrows) attached by a broad base to the parietal pericardium (panel C). Sectioning of the gross specimen, which measured about 6.0×5.0×2.5 cm, showed the protruding portion of the mass was surrounded by a glistening capsule and showed some ruptured areas that were haemorrhagic. Haematoxylin and eosin-stained sections (panel D) showed that the tumour was composed of monomorphic spindle cells in herringbone fascicular pattern (×100 magnification), and mitosis was counted to be 2 per 10 high power field. The immunohistochemical stains excluded the diagnoses of leiomyosarcoma, malignant schwannoma, synovial sarcoma, solitary fibrous tumour, and mesothelioma. The tumour was diagnosed as a malignant fibrosarcoma. The patient made an uneventful postoperative recovery and additional chemotherapy was planned.

Acknowledgments

This article has been adapted from Yoo S-Y, Park C-B, Cheong S S. Primary pericardial malignant fibrosarcoma presenting as sudden onset of substernal pain Heart 2008;94:265