Asian Cardiovasc Thorac Ann 2007;15:e7-e8
© 2007 Asia Publishing EXchange Ltd
Primary Malignant Fibrous Histiocytoma of the Heart
Janusz Skarysz, PhD,
Dariusz Go
ka, PhD1,
Micha Krejca, PhD,
Andrzej Bochenek, PhD
Department of Cardiosurgery
1 Department of Pathomorphology, Medical University of Silesia, Katowice, Poland
For reprint information contact: Janusz Skarysz, PhD, Tel: 48 32 359 8640, Fax: 48 32 252 7066, Email: jdoctor{at}wp.pl, 1st Department of Cardiosurgery, Medical University of Silesia, ul. Zioowa 45-47, 40-635 Katowice, Poland.
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ABSTRACT
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A 72-year-old male diagnosed with heart failure and dyspnea was found to have a lesion in his right atrium. Histological examination of the tumor revealed it to be a malignant fibrous histiocytoma. The patient’s management and subsequent follow-up are presented.
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INTRODUCTION
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Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma of adulthood, however its incidence in the heart is very rare (3%)1. It typically develops in the left atrium. A 72-year-old man who was initially examined by echocardiography for suspected chronic cardiac aneurysm exhibited left atrial MFH. The patient’s management and follow-up are presented.
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CASE REPORT
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A 72-year-old man was admitted to Silesian Heart Center from a municipal hospital on June 11th 2001 with symptoms of congestive heart failure. He had been experiencing increased dyspnea on exertion. These problems had begun several months before admission to the hospital. On physical examination peripheral edema and liver enlargement (hepatomegaly) were found. He had no fever and no lymphadenopathy. His arterial blood pressure was 140/90 mm Hg with a pulse rate of 92 beats·min–1. There were no abnormalities on laboratory blood and urine investigation. Chest X-Ray showed an enlarged right atrium. Transthoracic echocardiography revealed a tumor measuring 54 x 22 mm in the right atrium penetrating through the tricuspid valve to the right ventricle, suggestive of myxoma or thrombosis of the right atrium. The presence of the tumor was confirmed by computerized tomography (CT) and magnetic resonance (MR) scans. In addition, the MR scan revealed tumor penetration to the inferior vena cava (Figure 1
). No lymphadenopathy in the chest or abdomen was detected on CT or MR scan.
The patient was operated on via a median sternotomy. The pericardium was opened and some fluid was evacuated. The patient was connected to cardiopulmonary bypass by cannulating the ascending aorta, the superior and inferior vena cava. The patient was cooled to 32°C, the ascending aorta was cross clamped, and the heart was arrested by the infusion of cold blood cardioplegia 4:1 to the root of the aorta. The right atrium was opened revealing the yellowish mass of the tumor penetrating through the tricuspid valve to the right ventricle and inferior vena cava. Total resection of the complete tumor mass was not possible via this surgical access. The patient was subsequently cooled to deep hypothermia of 26°C, and the circulation arrested for 20 minutes. The cannula was then removed from the inferior vena cava and the entire tumor mass was resected.
On pathological examination, the hematoxylineosin stained sections of the surgical specimen showed an extremely pleomorphic, highly cellular tumor with a sarcomatous pattern consisting of anaplastic oval, polygonal, and multinucleated giant cells of high mitotic index (10/10 HPF). The cells were arranged haphazardly in solid sheets with accompanying necrotic areas involving up to 20% of the tumor (Figure 2). Abnormal mitotic figures were commonly seen. Immunohistochemical examination revealed focal positive reaction for CD68 and actin. The tumor was diagnosed as "malignant fibrous histiocytoma".
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Figure 2. Histological features of malignant fibrous histiocytoma; anaplastic tumor cells haphazardly arranged in solid sheets with prominent bizarre nuclei. (Hematoxylin and eosin stain x 160).
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The patient was discharged from hospital 2 weeks after surgery without adjuvant chemo- or radiotherapy. On follow-up, 39 months after hospitalization, the patient was still alive without major respiratory or circulatory insufficiency.
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DISCUSSION
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Malignant fibrous histiocytoma is a common sarcoma of late adulthood with a definite male predominance. Despite various possible primary localizations, its occurrence in the heart is exceptional.2,3 The suggested etiologic factor of MFH is radiation. In our case, however, the patient did not report any therapeutic or incidental irradiation. The histopathological diagnosis is primarily based on light microscopic features, with immunohistochemical evaluation playing only an ancillary role.4 Clinical symptoms, especially early in the course of the disease are non-specific, and in most cases include heart failure, dyspnea, and arrhythmia.5,6 Among methods used in the evaluation of cardiac tumors, echocardiography plays an important role.5 The tumor growth is usually infiltrating and aggressive with late clinical manifestations. The treatment of choice is surgical resection with eventual adjuvant chemotherapy, however prognosis, even with treatment, is generally poor.7 Due to the advanced tumor stage at diagnosis, most therapeutic regimens are mainly palliative.8
Our case indicates that despite a relatively large tumor size and the unfavorable location in terms of radical excision, wide surgical resection is the optimal treatment in attempt to prolong survival and improve quality of life.
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REFERENCES
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ABSTRACT
INTRODUCTION
