Primary Cardiac Myxofibrosarcoma Research Articles

Primary cardiac myxofibrosarcoma of the left atrium and pericardium: a case report

BackgroundPrimary cardiac myxofibrosarcoma is rare and commonly occurs in the left atrium. Myxofibrosarcoma is aggressive and has a high mortality rate due to its high rate of recurrence. Complete surgical resection is considered important; however, effective treatment options have not been established.Case presentationWe report the case of a 75-year-old woman who developed a myxofibrosarcoma spreading to the left atrium and pericardium. We performed surgical resection of the tumor to prevent sudden death due to mitral valve obstruction or cerebral infarction due to embolism of the scattered mass. However, we were unable to complete the resection of the tumors. The patient developed brain metastasis 2 months after surgery and eventually died due to brain hemorrhage 3 months after surgery.ConclusionsIn this report, we described a rare case of primary cardiac myxofibrosarcoma located not only in the left atrium but also in the pericardium. Considering preoperative laboratory findings, surgical and adjuvant therapy, and the patient’s wishes are important for the best therapeutic course for an individual.

Primary cardiac myxofibrosarcoma with osteoid differentiation mimicking a left atrial myxoma: A rare entity

Cardiac myxofibrosarcoma (MFS) is an uncommon entity. It is among the most challenging conditions to diagnose due to its rarity, high variability, and non-specific findings. These tumors often simulate left atrial myxoma or mitral stenosis at clinical presentation. Although, the definitive diagnosis of cardiac tumors depends on histopathological examination, various imaging techniques are also useful to study tumor characteristics to plan an appropriate treatment strategy. Here we highlight a case of primary cardiac MFS of left atrium (LA) showing areas of transition to undifferentiated pleomorphic sarcoma (UPS) with bone or osteoid formation, which is extremely rare and not well described.<Learning objective: Primary cardiac myxofibrosarcoma (MFS) is a rare and aggressive cardiac tumor. It is often confused with benign myxoma, leading to a delay in initiation of treatment. This delay can often lead to poor clinical outcomes. Our study will guide clinicians in early diagnosis, treatment, and counseling of patients with this rare entity. Echocardiography, together with magnetic resonance imaging, histology, and immunohistochemistry are essential in the diagnosis of MFS.>

A RARE CASE OF PRIMARY CARDIAC MYXOFIBROSARCOMA

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Primary cardiac myxofibrosarcoma is a rare malignancy of mesenchymal origin which is associated with poor prognosis. Patients are generally asymptomatic in the earlier stages, however can present with life threatening hemodynamic instability. CASE PRESENTATION: Patient is a 25-year-old Caucasian male with history of asthma who presented with sudden onset of unresponsiveness, refractory shock and extensive bilateral pulmonary infiltrates. He was treated for bronchitis and pneumonia as outpatient on and off for two months prior to presentation. A transthoracic echocardiography showed large rounded mobile mass (5.5 cm by 4.5 cm) in left atrial cavity which was attached to anterior mitral leaflet causing severe mitral stenosis. CT showed extensive bilateral pulmonary consolidations. The patient was not deemed a candidate for surgical resection of the atrial mass. In the light of grave prognosis and failed aggressive medical therapy, family opted for comfort care measures. Autopsy of the left atrial mass showed low grade primary cardiac atrial myxofibrosarcoma. DISCUSSION: Myxofibrosarcoma most commonly occurs in the extremities but rarely can be seen in the heart. Primary cardiac myxofibrosarcoma is seen in the left atrium about 75% of the time [1]. Prognosis is poor because of its invasive nature and poor response to therapy including radiation, resection and chemotherapy [2]. Patients tend to be asymptomatic in the earlier stages. Clinical presentation is usually due to mass effect from the tumor. Tumor specifically located in the left atrium can cause obstruction of the mitral valve which manifests as symptoms of functional mitral stenosis leading to symptoms associated with heart failure. It can produce “tumor plop” (sound from tumor bounding around in the heart) and murmur similar to the mid-diastolic rumble of mitral stenosis [3]. This case is significant not only because of rarity of primary myxofibrosarcoma but also the clinical presentation and acute decompensation. Per our literature review, there are no reports of primary cardiac myxofibrosarcoma that presented with acute respiratory distress syndrome (ARDS). Our patient’s acute decompensation is likely synergistic result of cardiogenic shock from functional mitral stenosis (tumor mass effect) as well as underlying septic shock from pneumonia. CONCLUSIONS: Primary cardiac myxofibrosarcoma is a rare tumor which is usually detected when there are apparent hemodynamic changes form the mass effect, at which time treatment is limited given its extensive spread. Palliative care is generally the final outcome. Reference #1: Ujihira, Kosuke et al. “A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure” Journal of cardiothoracic surgery vol. 11,1 95. 7 Jul. 2016. Reference #2: Lazaros GA, et al. Primary myxofibrosarcoma of the left atrium: case report and review of the literature. Angiology. 2008;59(5):632–635. Reference #3: Vaideeswar, P. Butany, JW. (Feb 2008). "Benign cardiac tumors of the pluripotent mesenchyme". Semin Diagn Pathol. 25 (1): 20–8. DISCLOSURES: No relevant relationships by Bishal Bhandari, source=Web Response No relevant relationships by Cameron Koester, source=Web Response No relevant relationships by Abhishek Kalidas Kulkarni, source=Web Response No relevant relationships by Manjari Regmi, source=Web Response

Primary cardiac myxofibrosarcoma: case report, literature review and pooled analysis

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.

Primary cardiac myxofibrosarcoma: a case report and review of the literature.

Primary myxofibrosarcoma of the heart is a rare malignancy. To the best of our knowledge, no more than 17 cases have been reported since 1963. We report the case of a 42-year-old man who presented with dyspnea and palpitation of one month's duration. Echocardiography and enhanced computed tomography revealed a large tumor in the left atrium. The patient underwent palliative excision of the tumor and histopathological analysis revealed it to be a low-grade myxofibrosarcoma. The patient developed a bone metastasis two years later and is still alive 26 months after the cardiac surgery. Primary cardiac myxofibrosarcoma involves predominantly the left atrium and the most common symptom induced by the tumor is dyspnea. Disease outcome is often disappointing despite aggressive postoperative radiotherapy and chemotherapy.

Primary Cardiac Myxofibrosarcoma: A Case Report and Review of the Literature

Aims and background Primary myxofibrosarcoma of the heart is a rare malignancy. To the best of our knowledge, no more than 17 cases have been reported since 1963. Methods and study design We report the case of a 42-year-old man who presented with dyspnea and palpitation of one month's duration. Echocardiography and enhanced computed tomography revealed a large tumor in the left atrium. Results The patient underwent palliative excision of the tumor and histopathological analysis revealed it to be a low-grade myxofibrosarcoma. The patient developed a bone metastasis two years later and is still alive 26 months after the cardiac surgery. Conclusions Primary cardiac myxofibrosarcoma involves predominantly the left atrium and the most common symptom induced by the tumor is dyspnea. Disease outcome is often disappointing despite aggressive postoperative radiotherapy and chemotherapy.