Cardiac Malignant Fibrous Histiocytoma Research Articles

Primary cardiac malignant fibrous histiocytoma with vulvar metastases: A case report.

Malignant fibrous histiocytoma (MFH) occurring in the heart is rare. To the best of our knowledge, the current study was the first to report a case of left atrial MFH metastasizing to the vulva. A 37-year-old female presenting with a history of exertional dyspnea underwent echocardiography, which revealed a mass in the left atrium, with a short-wide stalk attached to the posterior wall, extending into the pericardium. Furthermore, the mass resulted in severe mitral stenosis during diastole. The tumor was excised together with a section of the left atrial wall. The initial diagnosis was a left atrial myxoma. However, post-operative frozen section histopathology results indicated a diagnosis of MFH. Following surgery, the patient received chemotherapy, however, vulvar metastases and left pleural effusions were identified after 1 month. Following surgical excision of the vulvar tumor and left thoracentesis, the patient began adjuvant chemotherapy. However, the patient succumbed due to local recurrence 6 months later.

Primary cardiac malignant fibrous histiocytoma with abdominal wall metastasis

We present a rare case of cardiac malignant fibrous histiocytoma (MFH; undifferentiated pleomorphic sarcoma); to date, fewer than 100 cases of cardiac MFH have been reported. In this case, transthoracic echocardiography revealed cardiac tumors in the left atrium (LA) of a 53-year-old woman with a 3-month history of worsening dyspnea; the largest tumor was found to protrude through the mitral valve in diastole, causing stenosis. Three of the four tumors were resected during emergency surgery; however, the residual tumor extension into the left pulmonary vein could not be removed. Histological findings of the resected tumors, such as organized thrombus and myxomatous tissue changes, indicated that the tumors were benign. After 3 months, the patient underwent total resection for a small mass that developed on her right abdominal wall, which was revealed histologically to be MFH; additionally, the residual mass in the LA had enlarged progressively. After undergoing radiation therapy without further surgery, she died of cerebral bleeding 6 months after cardiac surgery. Postmortem examination revealed that the tumor in the LA was an MFH. Thus, cardiac MFH should be considered as a differential diagnosis for tumors on the posterior wall of the LA.<Learning objective: Primary cardiac malignant fibrous histiocytoma (MFH), which is easily mistaken for atrial myxoma, is a rare type of cardiac sarcoma. MFH occurs most commonly on the posterior wall of the left atrium (LA), and total resection is currently the only effective therapy; however, the prognosis is poor. Therefore, a high level of suspicion is required to facilitate early diagnosis. Cardiac MFH should be considered as a differential diagnosis for tumors on the posterior wall of the LA.>

Primary pleomorphic malignant fibrous histiocytoma of the heart

Primary pleomorphic malignant fibrous histiocytoma of the heart is rare. The present study was performed to study the clinical and pathological features of the disease. We describe two rare cases of primary cardiac malignant fibrous histiocytoma and review the published individual data of the patients. Both patients complained of dyspnea, and underwent palliative tumor resection. However, they died several months after surgery. A thorough literature review with clinical presentations, diagnostic features, treatment, and outcomes was done. We have for the first time analyzed the factors related to the survival of malignant fibrous histiocytoma. It is usually difficult to make an appropriate preoperative diagnosis. Despite complete surgical resection and aggressive chemotherapy and radiotherapy, the prognosis is still poor.

Non-diagnosed primary malignant cardiac tumor as a cause of sudden death in a 52-year-old man. A case report with medico-legal implications

We describe the case of a malignant fibrous histiocytoma (MFH) of the heart, presenting with an obstinate cough and clinically misdiagnosed as adult respiratory distress syndrome, in a 52-year-old man. He was diagnosed as pulmonary tuberculosis four years ago. Although his tuberculosis was believed to have cured, he often went to hospital for cough and palpitation. Echocardiogram was conducted only once on the patient four years ago but no abnormalities were found. During the day his condition deteriorated. He went to the emergency department, where he was given intravenous drugs against cough and was sent home. On the way back, his condition deteriorated dramatically so that his son drove back to the emergency room, where he collapsed and sustained cardiac arrest; resuscitation efforts were unsuccessful. Autopsy revealed a large tumor located in the left atrium. Histological and immunohistochemical investigations of the mass established the diagnosis of primary cardiac MFH. Sudden cardiac death, caused by the MFH was ascertained as the cause of death. The morphological and pathological findings are presented, the difficulty to diagnose primary cardiac MFH and the medico-legal implications are discussed.

Primary malignant fibrous histiocytoma of the heart

We describe a case of a primary cardiac malignant fibrous histiocytoma in the left atrium of a 72-year-old man. Local recurrences appeared twice, at 2 and 14 months after initial tumor resection, and were managed surgically. The patient died of pancreatic metastasis 22 months after the initial diagnosis. The prognosis for MFH of the heart is poor despite aggressive surgical treatment.

Recurrent Malignant Fibrous Histiocytoma of the Right Atrium with Extracardiac Extension

4Department of Pathology, Soonchunhyang University Hospital Bucheon, Bucheon, Gyunggi, 420-767, Korea. primary cardiac malignant fibrous histiocytoma (MFH) of the right atrium was diagnosed in a 34year-old woman admitted to our hospital with dyspnea. Pathohistological examination confirmed MFH. The tumor recurred rapidly, and a second operation was not performed. A CT chest scan revealed a large mass in the right atrium with extracardiac extension. The patient was treated with chemotherapy using a combined drug regimen. Despite hemodynamic impairment and extracardiac extension, adjuvant chemotherapy promoted partial remission. We report a case of recurrent primary cardiac MFH with extracardiac extension and successful chemotherapeutic management. Primary cardiac tumors are uncommon and most are benign. Malignant tumors account for only 25% of all primary cardiac tumors [1, 2]. Most malignant cardiac tumors are angiosarcomas. Malignant fibrous histiocytomas are rare primary cardiac tumors [3–6]. We report on a case of recurrent primary cardiac MFH with extracardiac extension and successful chemotherapeutic management.

Complete excision of primary cardiac malignant fibrous histiocytoma involving the left atrial free wall and mitral annulus by modified autotransplantation

Complete excision of primary cardiac malignant fibrous histiocytoma involving the left atrial free wall and mitral annulus by modified autotransplantation

Chemotherapeutic potential of plant alkaloids and multidrug resistance mechanisms in malignant fibrous histiocytoma of the heart.

Primary malignant fibrous histiocytoma (MFH) of the heart is a rare and highly malignant soft tissue tumor, which is largely resistant to conventional chemotherapy and radiotherapy. Therefore, we analyzed growth inhibitory effects of different chemotherapeutic agents and mechanisms of drug resistance in the recently established cell line MFH-H derived from a human primary cardiac MFH. The growth inhibitory effects of etoposide, vincristine, and paclitaxel were tested using the MTT assay. The expression and function of multidrug resistance-related proteins, i.e. the P-glycoprotein, the multidrug resistance-associated protein (MRP) and the lung resistance-related protein (LRP) were determined by FACScan and functional assays of cellular drug efflux. The concentration required for a 50% inhibition of growth (IC50) was 0.001 microM for etoposide and 0.035 microM for vincristine. Paclitaxel dissolved in Cremophor EL/ethanol inhibited the cell growth of MFH-H cells more intensively (IC50: 0.27 microM) than paclitaxel dissolved in DMSO (IC50: 11.09 microM) suggesting that Cremophor EL is contributing to the inhibitory effects of paclitaxel. The response of MFH-H to etoposide, vincristine and paclitaxel/Taxol could not be predicted by the expression and function of P-glycoprotein, MRP and LRP. This study demonstrates that etoposide and to a lesser extent vincristine can effectively inhibit the growth of MFH-H cells, irrespective of the multidrug resistance phenotype. MFH-H cells are relatively insensitive to paclitaxel dissolved in DMSO, in contrast to paclitaxel dissolved in Cremophor EL/ethanol indicating that the diluent Cremophor contributes to the antiproliferative effects of the taxane paclitaxel.

A Case of Primary Malignant Fibrous Histiocytoma of the Heart With a Left-to-Right Atrial Shunt

A previously healthy 64-year-old woman attended our hospital with chest pain, facial edema, and general fatigue. A chest radiograph revealed cardiomegaly, small bilateral pleural effusions, and hilar congestion--findings that improved after early therapy with furosemide and methyldigoxin. A chest radiograph recorded 7 years earlier had revealed no dilation of cardiac shadow. There were no findings suggesting atrial septal defect (ASD) or valvular heart disease. Echocardiography revealed a tumor-like mass adhering to the posterior wall of the left atrium. Color-flow Doppler echocardiography revealed a left-to-right shunt at the atrial level. The Qp/Qs ratio as measured by cardiac catheterization was 2.0. Coronary angiography revealed abnormal dilated arteries from the atrioventricular nodal branch and several feeding arteries from the left circumflex branch. We hypothesized that the left-to-right shunt could be due to the tumor, which extended to the rim of the patent foramen ovale, or to the very small, previously unrecognized, ASD. This patient died 6 months after her first admission and an autopsy was performed. Light microscopic examination of the tumor revealed spindle-shaped fibroblast-like cells arranged in a storiform or fascicular pattern. The immunohistochemical findings were consistent with malignant fibrous histiocytoma (MFH). In the literature, left-to-right shunt at the atrial level has not been reported in patients with cardiac MFH.

Cardiac malignant fibrous histiocytoma metastasizing to the brain: Development of multiple neoplastic cerebral aneurysms

We report a case in which bilateral occipital brain metastases and neoplastic cerebral aneurysms developed from primary cardiac malignant fibrous histiocytoma. The origin of metastases was confirmed at autopsy. The clinical presentation, radiographic features, and autopsy findings are presented along with a histopathologic analysis of the tumor.