“…Due to aggressive behavior of the tumor, the presence of hemorrhage within the tumor contributed to focal dark-red color [ 37 ]. Different from cardiac myxoma, primary cardiac myxofibrosarcoma often showed irregular in shape, such as nodular, polypoid, papillary, bilobed, or multilobed [ 16 , 17 , 24 ]. Immunohistochemical stains were crucial importance in distinguishing primary cardiac myxofibrosarcomas from other spindle-cell malignancies.…”
BackgroundPrimary cardiac myxofibrosarcoma is a very rare cardiac malignancy. The majority of publications are limited to case reports. No pooled analyses of primary cardiac myxofibrosarcoma cases are available. Little clinical features and outcome patterns are acknowledged. The purpose of this study is to identify the clinical characteristics and prognostic factors of primary cardiac myxofibrosarcoma.Case presentationA case report of primary cardiac myxofibrosarcoma was presented, and a review of English language literatures of primary cardiac myxofibrosarcomas were performed electronically. Demographics, clinicopathologic data, therapy and follow-up were summarized. The median survival time and the mean survival time were calculated by Kaplan-Meier method. Survival distribution and overall survival were figured by log-rank test and cox proportional hazards models. We present a case, and retrospectively analyzed additional 30 patients derived from 24 isolated articles. The cohort consisted of 18 male and 13 female patients. The age was 41.87 ± 17.89 years. Some common features were found in clinical presentations, pathologic features, treatments and outcome patterns of primary cardiac myxofibrosarcoma. There were special features in echocardiography, histological and immunohistochemical examinations, which should be considered in diagnosis of primary cardiac myxofibrosarcoma. The median survival time/mean survival time (MST) was 14/32.66 months. The median survival time/mean survival time (MST) was 14/32.66 months. Compared to the other groups, the following groups had shorter survival characteristics, including age ≥ 40 years (14/11.79 months), female (14/26.26 months), mass diameter ≥ 40 mm (14/14.64 months), high-grade (2/11.81 months), and no post-treatment (14/28.09 months). Statistical analyses revealed that primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Tumors ≥ 40 mm in size (P = 0.055, HR = 6.79) or with high-grade (P = 0.063, HR = 11.45) had significantly worse prognosis.ConclusionsPrimary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Echocardiography, together with histological method should be considered in ordinary diagnosis. Tumors ≥ 40 mm in size or with high-grade had significantly worse prognosis, which should be early diagnosed and treated with rational surgery.
“…Due to aggressive behavior of the tumor, the presence of hemorrhage within the tumor contributed to focal dark-red color [ 37 ]. Different from cardiac myxoma, primary cardiac myxofibrosarcoma often showed irregular in shape, such as nodular, polypoid, papillary, bilobed, or multilobed [ 16 , 17 , 24 ]. Immunohistochemical stains were crucial importance in distinguishing primary cardiac myxofibrosarcomas from other spindle-cell malignancies.…”
BackgroundPrimary cardiac myxofibrosarcoma is a very rare cardiac malignancy. The majority of publications are limited to case reports. No pooled analyses of primary cardiac myxofibrosarcoma cases are available. Little clinical features and outcome patterns are acknowledged. The purpose of this study is to identify the clinical characteristics and prognostic factors of primary cardiac myxofibrosarcoma.Case presentationA case report of primary cardiac myxofibrosarcoma was presented, and a review of English language literatures of primary cardiac myxofibrosarcomas were performed electronically. Demographics, clinicopathologic data, therapy and follow-up were summarized. The median survival time and the mean survival time were calculated by Kaplan-Meier method. Survival distribution and overall survival were figured by log-rank test and cox proportional hazards models. We present a case, and retrospectively analyzed additional 30 patients derived from 24 isolated articles. The cohort consisted of 18 male and 13 female patients. The age was 41.87 ± 17.89 years. Some common features were found in clinical presentations, pathologic features, treatments and outcome patterns of primary cardiac myxofibrosarcoma. There were special features in echocardiography, histological and immunohistochemical examinations, which should be considered in diagnosis of primary cardiac myxofibrosarcoma. The median survival time/mean survival time (MST) was 14/32.66 months. The median survival time/mean survival time (MST) was 14/32.66 months. Compared to the other groups, the following groups had shorter survival characteristics, including age ≥ 40 years (14/11.79 months), female (14/26.26 months), mass diameter ≥ 40 mm (14/14.64 months), high-grade (2/11.81 months), and no post-treatment (14/28.09 months). Statistical analyses revealed that primary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Tumors ≥ 40 mm in size (P = 0.055, HR = 6.79) or with high-grade (P = 0.063, HR = 11.45) had significantly worse prognosis.ConclusionsPrimary cardiac myxofibrosarcomas were more likely to present with local recurrences and dismal metastases. Echocardiography, together with histological method should be considered in ordinary diagnosis. Tumors ≥ 40 mm in size or with high-grade had significantly worse prognosis, which should be early diagnosed and treated with rational surgery.
A case report of a 28-year-old woman with malignant fibrous histiocytoma (MFH) of the left atrium is presented, and the six previous reports of this rare cardiac tumor are reviewed. A tendency for malignant fibrous histiocytoma of the heart to occur in the left atrium of young women is suggested; this sarcoma's usual location is in the soft tissue of elderly men. The apparent predilection for the left atrium is unique among cardiac malignancies. Careful pathologic study is necessary to differentiate the uniformly fatal MFH of the heart from the more common benign atrial myxoma. Cancer 59:1026-1031. 1987. ALIGNANT FIBROUS HISTIOCYTOMA (MFH) is one M of the most common soft tissue sarcomas,' but its presentation as a primary cardiac tumor is distinctly unusual. The current case illustrates two important points regarding MFH of the heart: (1) it may be confused clinically and histologically with benign atrial myxoma (BAM), the most common cardiac tumor; and (2) it occurs in a younger, predominantly female population in con-tradistinction to its occurrence in the soft tissue where it is most commonly seen in elderly men.2,3 Case Report A 28-year-old Hispanic woman in her third trimester of pregnancy presented to her obstetrician in September 1983, with a 1-week history of shortness of breath, orthopnea, and cough productive of purulent sputum. She reported increasing fatigue for 2 months. She was markedly dyspneic at rest with blood pressure of 100/70 and a heart rate of 130/minute. The first heart sound was loud, and the second was physiologically split. A short Grade II/VI systolic murmur was heard at the lower left sternal border, and a I/VI diastolic rumble was audible at the apex and lower left sternal border, A gravid uterus of 7 months gestation was noted. The hematocrit was 3190, leukocyte count From the was 12,000/mm3 with a left shift. A few ovalocytes and schis-tocytes were seen on the peripheral smear. Sputum and blood culture showed no growth. A chest x-ray revealed bilateral in-filtrative changes consistent with congestion, although the heart size was within normal limits. An electrocardiogram (ECG) revealed sinus tachycardia. Two-D and M-Mode echocardiography showed the right atrial, right ventricular, and left ventricular cavities to be normal in size. The left atrium was enlarged (4.6 cm); the two-D films revealed a mass in the left atrium which appeared to arise from the posterior wall immediately above the mitral valve. The patient was treated for a time with diuretics, bed rest, and sodium restriction, but because of progressive cardiac de-compensation a caesarian section was performed at 37 weeks gestation. One week later three large atrial tumors were excised via a right anterior thoracotomy, all three attached to the posterior atrial wall. These were pedunculated, their bases separated by normal-appearing endocardium. The tumors were removed at their bases, and adjacent endocardium was resected. Four months later the patient again presented with symptoms of congestive heart failure. An echocar...
Primary soft tissue sarcoma of the heart is encountered infrequently in clinical practice. Treatment is reported of an intracardiac malignant fibrous histiocytoma, consisting of maximal surgical resection followed by 5600 cGy of conventionally fractionated radiation therapy. Transesophageal echocardiograms done during follow‐up were useful in assessing tumor control. Aspects of patient care are discussed in conjunction with a review of the available literature. Cancer 1992; 69:956–961.
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