Ann Thorac Surg 2008;86:646-649. doi:10.1016/j.athoracsur.2008.02.040
© 2008 The Society of Thoracic Surgeons
Case Reports
Primary Pericardial Malignant Fibrous Histiocytoma Causing Cardiac Tamponade
Seiji Matsukuma, MDa,*,
Hiroshi Yamaguchi, MDa,
Masayoshi Hamawaki, MDa,
Masahiro Ito, MDb,
Yohjiro Matsuoka, MDc
a Department of Cardiovascular Surgery, National Hospital Organization Nagasaki Medical Center, Omura City, Nagasaki, Japan
b Department of Pathology, National Hospital Organization Nagasaki Medical Center, Omura City, Nagasaki, Japan
c Department of Radiology, National Hospital Organization Nagasaki Medical Center, Omura City, Nagasaki, Japan
Accepted for publication February 14, 2008.
* Address correspondence to Dr Matsukuma, Department of Cardiovascular Surgery, National Hospital Organization Nagasaki Medical Center, 2-1001-1 Kubara, Omura City, Nagasaki, 856-8562, Japan (Email: matsukuma{at}nmc.hosp.go.jp).
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Abstract
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We describe herein an extremely rare case of a large primary pericardial malignant fibrous histiocytoma causing a cardiac tamponade that occurred in a 72-year-old woman. The clinical, radiographic, and pathologic features are reported here together with a brief review of the literature.
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Introduction
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Primary malignant tumors of the pericardium are extremely rare. A malignant fibrous histiocytoma (MFH) of the heart is a rare condition with an unfavorable prognosis. We describe an extremely rare case of a large primary pericardial MFH causing a cardiac tamponade.
A 72-year-old woman was admitted with severe dyspnea. A chest x-ray film showed marked cardiomegaly and bilateral pleural effusion. Computed tomography (CT) confirmed bilateral pleural and pericardial effusions and hilar lymphadenopathy, but no other abnormalities (eg, lung cancer, breast cancer, or aortic dissection) were noted (Fig 1A). Acute pericarditis caused by an infection or sarcoidosis was suspected as a differential diagnosis on computed tomography. Cytological study and culture of the aspirated serous pleural effusion and hemorrhagic pericardial effusion were nonspecific. Re-accumulation of the pericardial fluid did not occur. The patient was discharged from the hospital on the 14th day. Twenty days after discharge she had dyspnea again, and echocardiographic and computed tomographic scans revealed a large mass in the pericardium (Fig 1B) that did not exist at the time of her first admission. Magnetic resonance imaging revealed a large oval-shaped mass (10 x 10 x 5 cm) with enhancement of the wall and part of the contents located under both ventricles. There appeared to be a plane between the tumor and myocardium on magnetic resonance imaging. Little pericardial effusion existed in the pericardial space, and the mass itself compressed both ventricles and caused an upward shift of the heart (Figs 2A and 2B). Because congestive heart failure progressed rapidly and became uncontrollable, we decided to do emergency surgery to relieve the cardiac tamponade and to establish a diagnosis.
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Fig 1. (A) Computed tomographic image of first admission reveals a large circumferential pericardial effusion. Mass lesions were absent. (B) Computed tomographic image taken 34 days after the first admission reveals mass lesion (arrows).
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Fig 2. (A) T2-weighted spin echocardiographic image shows a large nonhomogenous mass pushing the ventricles upward (arrows). (B) T1-weighted gadolinium-enhanced magnetic resonance imaging demonstrates the mass with an enhanced wall and partially enhanced contents (white arrows). The mass does not extend over the atrioventricular groove (black arrows).
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Surgery was carried out through a median sternotomy. The parietal pericardium had become thick and hard. The mass in the pericardial sac had a thin, white wall and the contents consisted of a gray-white nodular mass and a yellow myxoid material (Fig 3A). Because the tumor had adhered firmly to the whole inferior surface of both ventricles and the pericardium, complete resection was not accomplished. The pericardium facing both pleural cavities was widely excised to prevent an early recurrence of a cardiac tamponade. The patient recovered smoothly, and she was immediately sent to the department of oncology for chemotherapy. However, 2 months later, the combination of chemotherapy (consisting of gemcitabine hydrochloride and doxorubicin hydrochloride) proved to be ineffective, and the patient died of progressive constrictive pericarditis.
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Fig 3. (A) A part of resected yellow myxoid material. (B) Microscopic appearance of the tumor showing a striform pattern of spindle and foamy cells (hematoxylin-eosin stain, x 100). (C) Myxomatous area of the tumor with a giant cell (arrow) (hematoxylin-eosin stain, x 120).
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Histopathology showed pleomorphic proliferations (in a striform pattern, a myxomatous pattern, and an alveolar pattern) of atypical spindle or oval cells admixed with scattered bizarre giant cells in a well-vascularized fibromyxoid stroma with necrosis and hemorrhaging (Figs 3B and 3C). Mitotic figures were readily encountered (more than 20 mitosis per 10 high-power fields). Immunohistochemical staining for desmins, myoglobin, cytokeratin, CD34, S-100, and carcinoembryonic antigen were all negative. However, the tumor cells were positive for vimentin and were very focally positive for alpha-smooth muscle actins and calretinin. Histopathologically, no specific line of differentiation (other than focal potential myofibroblastic or mesothelial differentiation) was identified, and the tumor was diagnosed as a pleomorphic MFH (ie, undifferentiated high-grade pleomorphic sarcoma).
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Comment
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The MFH has been regarded as the most common soft tissue sarcoma of older adults [1]. Malignant fibrous histiocytoma typically occurs on the extremities and the retroperitoneum and consists of a multi-lobulated gray-white, fleshy mass. Approximately 25% of MFHs are highly myxoid and thus grossly resemble myxoid liposarcomas [1].
Primary MFH of the heart accounts for only 1.6 to 1.8% of primary cardiac tumors [2] and commonly arises in the left atrium [3, 4]. To date, nearly 50 cases of cardiac MFHs have been reported. However, among these there were only four tumors found to be located in the pericardium [3–7].
Survival of cardiac MFH patients ranged from less than 1 month to 6 years after diagnosis, with a mean survival period of approximately 1 year [3]. All five MFHs located in the pericardium, including our case, died within 6 months after diagnosis. The prognosis in cases of primary pericardial malignancy is generally poor, because surgical resection is often incomplete, and chemotherapy and radiotherapy often yield only temporary improvement. We believe that early diagnosis prior to deep or wide invasion and metastasis is important, because it might increase the possibility of a complete resection and transplantation.
In conclusion, our case of MFH can be considered particularly unique because of its large dimensions, its extremely fast growth rate, its rare growth location, and its likely origin (the mesenchymal tissue of visceral pericardium).
In most cases of a pericardial tumor, the diagnosis is delayed until evidence of cardiac compression or tamponade appears. The unreliability of a cytological examination of pericardial fluid is one of the reasons behind a late diagnosis, as was true in our case.
Development of early diagnostic methods and effective therapies are required to improve the prognosis of patients with pericardial tumors, including MFH.
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References
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- Weiss SW, Enzinger FM. Malignant fibrous histiocytoma: an analysis of 200 cases Cancer 1978;41:2250-2266.[Medline]
- Korbmacher B, Doering C, Schulte HD, et al. Malignant fibrous histiocytoma of the heart: case report of a rare left-atrial tumor Thorac Cardiovasc Surg 1992;40:303-307.[Medline]
- Okamoto K, Kato S, Katsuki S, et al. Malignant fibrous histiocytoma of the heart: case report and review of 46 cases in the literature Intern Med 2001;40:1222-1226.[Medline]
- Dorobantu M, Fruntelate A, Constantinescu D, et al. Primary left heart malignant fibrous histiocytoma Eur J Echocardiogr 2005;6:225-227.[Abstract/Free Full Text]
- Fujio A, Kitano M, Asakura S, Matsui T. A case of malignant histiocytoma of the epicardium Nippon Kyobu Geka Gakkai Zasshi 1986;34:267-271.[Medline]
- Wilk W, Janicki K. Primary intrapericardial malignant fibrous histiocytoma: a case report Pol J Pathol 1998;49:43-46.[Medline]
- Tanprasert P, Vidhayasarronyut S, Dharamadhach A, Suchatlampong V. A rare case of pericardial malignant fibrous histiocytoma J Med Assoc Thai 1981;64:473-479.[Medline]
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Abstract
Introduction
