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Left ventricular malignant fibrous histiocytoma
Clinical Imaging 31 (2007) 422 – 424
Left ventricular malignant fibrous histiocytoma Ta-Jung Chung4, Lili Cheng, Chin-Ying Yu Department of Radiology, National Cheng-Kung University Hospital, Tainan 704, Taiwan Received 22 January 2007; accepted 15 March 2007
Abstract Primary malignant fibrous histiocytoma (MFH) of the heart is rare. A 39-year-old male with symptoms of infectious endocarditis was transferred to our hospital. Transesophageal echocardiography and magnetic resonance imaging showed a mass in the left ventricle of heart with a short stalk attached to the lateral wall. He underwent open heart surgery, resecting the tumor. The pathologic diagnosis was MFH. D 2007 Elsevier Inc. All rights reserved. Keywords: Malignant fibrous histiocytoma; Heart neoplasm; Left ventricle
1. Case report A 39-year-old male patient was presented with complaint of fever, chills, watery diarrhea, abdominal pain, and lethargy. He had a history of chronic alcoholinduced pancreatitis and diabetes mellitus. He was admitted to Chia-Yi Veterans Hospital on May 8. Echocardiography showed a vegetation (22 cm) in the left ventricle. Abdominal computed tomography revealed a right renal infarction probably secondary to septic emboli. After a few days of antibiotics treatment, his condition improved slightly. He was then transferred to our hospital on May 13. On admission, the heart rhythm was regular. There were no murmurs on chest auscultation. Physical examination showed needle punctures over bilateral upper extremities and cellulitis with gangrene over the medial aspect of the right thigh. Antibiotics treatment was continued (according to the multiorganism bacteremia), including Klebsiella pneumoniae, Staphylococcus aureus, and Candida albicans, but he still suffered from spiking fever. Infectious endocarditis was
4 Corresponding author. Department of Radiology, National Cheng Kung University Hospital 138, Sheng-Li Road, Tainan City 704, Taiwan. Tel.: +886 6 2353535x2401; fax: +886 6 2766608. E-mail address: [email protected] (T.-J. Chung). 0899-7071/07/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.clinimag.2007.03.002
highly suspected. Debridement of his right thigh gangrene was performed on May 18th. Transesophageal echocardiography on May 19th revealed a left ventricular mass, about 21.8 cm in size, with a short stalk attaching to the lateral
Fig. 1. Axial T1-weighted image showed an isointense mass in the left ventricle.
T.-J. Chung et al. / Clinical Imaging 31 (2007) 422 – 424
423
Fig. 2. The mass showed hyperintensity to the myocardium on axial T2weighted image.
Fig. 4. Inhomogeneous Gd enhancement of the mass was found on the postcontrast axial T1-weighted fat-saturated image.
wall. Cardiac magnetic resonance imaging (MRI) was carried out on June 1st after his condition improved. The cardiac MRI showed a tumor, about 2.1cm in size, with isointensity on T1-weighted images, hyperintensity on T2-weighted images, and inhomogeneous Gd enhancement in the left ventricle (Figs. 1–4). Open heart surgery was performed on June 7th. A soft tissue tumor, measuring 2.61.81.8 cm, was resected. The micrograph showed that most part of the tumor was myxoid and hypocellular. The pleomorphic spindle cells were scattered in the central area with inflammatory cell infiltration. Mitotic figures were easily
found, and the muscle bundles were entrapped by the tumor. The spindle cells were positive for vimentin and smooth muscle actin. Stainings for desmin, S-100 protein, and calretinin were negative. A diagnosis of malignant fibrous histiocytoma (MFH) was made. The postoperative course was uneventful. The patient was afebrile. Neither radiotherapy nor chemotherapy was given. He discharged on June 15.
Fig. 3. The mass attached to the lateral wall of the left ventricle with a short stalk on long-axis T2-weighted image.
2. Discussion Primary cardiac sarcomas include angiosarcoma, osteosarcoma, fibrosarcoma, MFH, leiomyosarcoma, myxosarcoma, synovial sarcoma, neurofibrosarcoma, lymphosarcoma, reticulum cell sarcoma, and undifferentiated sarcoma. The first reported case of cardiac MFH was published by Shah et al in 1978 [1]. Primary MFH of heart is the second most common primary sarcoma, about 11.7% of cases reported [2]. Cardiac MFH affects middle-aged people ranging from 14–77 years old [1]. Most of the cardiac sarcomas tend to occur in the right side of the heart, although the cardiac MFH is predominantly located in the left atrium [1]. When the cardiac MFH is small, there are no clinical manifestations [3]. As the tumor grows, there are variable symptoms of the cardiac MFH according to the location of the tumor. The most frequent symptoms are shortness of breath, palpitation, or chest discomfort. In our case, the tumor was in the left ventricle and caused symptoms mimicking infectious endocarditis. The first choice of image modality is echocardiography for a noninvasive assessment of heart tumors, especially transesophageal echocardiography [4]. Computed tomography and magnetic resonance image are also of help.
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T.-J. Chung et al. / Clinical Imaging 31 (2007) 422 – 424
Definite diagnosis is still based on the pathologic findings. Surgical removal of tumor is the only method of curative therapy. Open heart surgery with an atriotomy under cardiopulmonary bypass is usually performed. Endoscopic tumor resection via aortotomy has been reported as an alternative approach [5]. The rate of surgical complication and mortality is low. Postoperative chemotherapy or radiotherapy has not been proven effective [2]. Although the prognosis of malignant cardiac tumor is poor [6] and the possibility of local recurrence and metastasis is high, the patients benefit from surgery to obtain histological information and to get improvement of symptoms. Heart transplantation may be considered a radical treatment of cardiac malignancy [7], but its indication is limited due to high potential of metastasis. The most common target organs of cardiac MFH metastasis are brain, lung, bone, and adrenal glands. Routine postoperation echocardiography and chest computed tomography follow-up are recommended for detecting early the local recurrence and metastasis.
References [1] Okamoto K, Kato S, Katsuki S, Wada Y, Toyozumi Y, Morimatsu M, Aoyagi S, Imaizumi T. Malignant fibrous histiocytoma of the heart: case report and review of 46 cases in the literature. Intern Med 2001;40(12):1222 – 6. [2] Toda R, Yotsumoto G, Masuda H, Sakata R, Umekita Y. Surgical treatment of malignant fibrous histiocytoma in the left atrium and pulmonary veins: report of a case. Surg Today 2002;32(3):270 – 3. [3] Schena S, Caniglia A, Agnino A, Caruso G, Ferlan G. Survival following treatment of a cardiac malignant fibrous histiocytoma. Chest 2000;118(1):271 – 3. [4] Hsieh PL, Lee D, Chiou KR, Kung MH, Lin SL, Liu CP, Chiang HT. Echocardiographic features of primary cardiac sarcoma. Echocardiography 2002;19(3):215 – 20. [5] Takahashi M, Yamamoto S, Saga T. Images in cardiovascular medicine Endoscopic resection of malignant fibrous histiocytoma in left ventricle. Circulation 1998;98(3):276 – 7. [6] Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69(2):387 – 95. [7] Harlamert HA, Moulton JS, Lewis W. Images in cardiovascular medicine Primary malignant fibrous histiocytoma of the heart treated with orthotopic heart transplantation. Circulation 1998;97(7):703 – 4.
Left ventricular malignant fibrous histiocytoma Ta-Jung Chung4, Lili Cheng, Chin-Ying Yu Department of Radiology, National Cheng-Kung University Hospital, Tainan 704, Taiwan Received 22 January 2007; accepted 15 March 2007
Abstract Primary malignant fibrous histiocytoma (MFH) of the heart is rare. A 39-year-old male with symptoms of infectious endocarditis was transferred to our hospital. Transesophageal echocardiography and magnetic resonance imaging showed a mass in the left ventricle of heart with a short stalk attached to the lateral wall. He underwent open heart surgery, resecting the tumor. The pathologic diagnosis was MFH. D 2007 Elsevier Inc. All rights reserved. Keywords: Malignant fibrous histiocytoma; Heart neoplasm; Left ventricle
1. Case report A 39-year-old male patient was presented with complaint of fever, chills, watery diarrhea, abdominal pain, and lethargy. He had a history of chronic alcoholinduced pancreatitis and diabetes mellitus. He was admitted to Chia-Yi Veterans Hospital on May 8. Echocardiography showed a vegetation (22 cm) in the left ventricle. Abdominal computed tomography revealed a right renal infarction probably secondary to septic emboli. After a few days of antibiotics treatment, his condition improved slightly. He was then transferred to our hospital on May 13. On admission, the heart rhythm was regular. There were no murmurs on chest auscultation. Physical examination showed needle punctures over bilateral upper extremities and cellulitis with gangrene over the medial aspect of the right thigh. Antibiotics treatment was continued (according to the multiorganism bacteremia), including Klebsiella pneumoniae, Staphylococcus aureus, and Candida albicans, but he still suffered from spiking fever. Infectious endocarditis was
4 Corresponding author. Department of Radiology, National Cheng Kung University Hospital 138, Sheng-Li Road, Tainan City 704, Taiwan. Tel.: +886 6 2353535x2401; fax: +886 6 2766608. E-mail address: [email protected] (T.-J. Chung). 0899-7071/07/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.clinimag.2007.03.002
highly suspected. Debridement of his right thigh gangrene was performed on May 18th. Transesophageal echocardiography on May 19th revealed a left ventricular mass, about 21.8 cm in size, with a short stalk attaching to the lateral
Fig. 1. Axial T1-weighted image showed an isointense mass in the left ventricle.
T.-J. Chung et al. / Clinical Imaging 31 (2007) 422 – 424
423
Fig. 2. The mass showed hyperintensity to the myocardium on axial T2weighted image.
Fig. 4. Inhomogeneous Gd enhancement of the mass was found on the postcontrast axial T1-weighted fat-saturated image.
wall. Cardiac magnetic resonance imaging (MRI) was carried out on June 1st after his condition improved. The cardiac MRI showed a tumor, about 2.1cm in size, with isointensity on T1-weighted images, hyperintensity on T2-weighted images, and inhomogeneous Gd enhancement in the left ventricle (Figs. 1–4). Open heart surgery was performed on June 7th. A soft tissue tumor, measuring 2.61.81.8 cm, was resected. The micrograph showed that most part of the tumor was myxoid and hypocellular. The pleomorphic spindle cells were scattered in the central area with inflammatory cell infiltration. Mitotic figures were easily
found, and the muscle bundles were entrapped by the tumor. The spindle cells were positive for vimentin and smooth muscle actin. Stainings for desmin, S-100 protein, and calretinin were negative. A diagnosis of malignant fibrous histiocytoma (MFH) was made. The postoperative course was uneventful. The patient was afebrile. Neither radiotherapy nor chemotherapy was given. He discharged on June 15.
Fig. 3. The mass attached to the lateral wall of the left ventricle with a short stalk on long-axis T2-weighted image.
2. Discussion Primary cardiac sarcomas include angiosarcoma, osteosarcoma, fibrosarcoma, MFH, leiomyosarcoma, myxosarcoma, synovial sarcoma, neurofibrosarcoma, lymphosarcoma, reticulum cell sarcoma, and undifferentiated sarcoma. The first reported case of cardiac MFH was published by Shah et al in 1978 [1]. Primary MFH of heart is the second most common primary sarcoma, about 11.7% of cases reported [2]. Cardiac MFH affects middle-aged people ranging from 14–77 years old [1]. Most of the cardiac sarcomas tend to occur in the right side of the heart, although the cardiac MFH is predominantly located in the left atrium [1]. When the cardiac MFH is small, there are no clinical manifestations [3]. As the tumor grows, there are variable symptoms of the cardiac MFH according to the location of the tumor. The most frequent symptoms are shortness of breath, palpitation, or chest discomfort. In our case, the tumor was in the left ventricle and caused symptoms mimicking infectious endocarditis. The first choice of image modality is echocardiography for a noninvasive assessment of heart tumors, especially transesophageal echocardiography [4]. Computed tomography and magnetic resonance image are also of help.
424
T.-J. Chung et al. / Clinical Imaging 31 (2007) 422 – 424
Definite diagnosis is still based on the pathologic findings. Surgical removal of tumor is the only method of curative therapy. Open heart surgery with an atriotomy under cardiopulmonary bypass is usually performed. Endoscopic tumor resection via aortotomy has been reported as an alternative approach [5]. The rate of surgical complication and mortality is low. Postoperative chemotherapy or radiotherapy has not been proven effective [2]. Although the prognosis of malignant cardiac tumor is poor [6] and the possibility of local recurrence and metastasis is high, the patients benefit from surgery to obtain histological information and to get improvement of symptoms. Heart transplantation may be considered a radical treatment of cardiac malignancy [7], but its indication is limited due to high potential of metastasis. The most common target organs of cardiac MFH metastasis are brain, lung, bone, and adrenal glands. Routine postoperation echocardiography and chest computed tomography follow-up are recommended for detecting early the local recurrence and metastasis.
References [1] Okamoto K, Kato S, Katsuki S, Wada Y, Toyozumi Y, Morimatsu M, Aoyagi S, Imaizumi T. Malignant fibrous histiocytoma of the heart: case report and review of 46 cases in the literature. Intern Med 2001;40(12):1222 – 6. [2] Toda R, Yotsumoto G, Masuda H, Sakata R, Umekita Y. Surgical treatment of malignant fibrous histiocytoma in the left atrium and pulmonary veins: report of a case. Surg Today 2002;32(3):270 – 3. [3] Schena S, Caniglia A, Agnino A, Caruso G, Ferlan G. Survival following treatment of a cardiac malignant fibrous histiocytoma. Chest 2000;118(1):271 – 3. [4] Hsieh PL, Lee D, Chiou KR, Kung MH, Lin SL, Liu CP, Chiang HT. Echocardiographic features of primary cardiac sarcoma. Echocardiography 2002;19(3):215 – 20. [5] Takahashi M, Yamamoto S, Saga T. Images in cardiovascular medicine Endoscopic resection of malignant fibrous histiocytoma in left ventricle. Circulation 1998;98(3):276 – 7. [6] Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69(2):387 – 95. [7] Harlamert HA, Moulton JS, Lewis W. Images in cardiovascular medicine Primary malignant fibrous histiocytoma of the heart treated with orthotopic heart transplantation. Circulation 1998;97(7):703 – 4.