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Background Myxofibrosarcomas (MFS) are a historically heterogeneous group of tumors that exhibit a propensity for local recurrence. The objectives of this study were to analyze the prognostic factors and outcomes of patients with MFS treated at a single institution. Methods We retrospectively reviewed the records of 69 consecutive patients with pathologically confirmed MFS of the extremities or superficial trunk who underwent surgery from August 1995 to November 2010. Clinicopathologic features, treatments, and patient outcomes were reviewed. Results Sixty-nine patients were identified, of whom 38 were men (55 %). The median age was 62 years. Sixty-four patients (93 %) presented with primary tumors, and 5 patients (7 %) presented with locally recurrent tumors. Median tumor size was 6.0 cm, and 44 patients (64 %) had grade 3 tumors (FNCLCC [Fédération Nationale des Centres de Lutte Contre le Cancer] classification). Margins were microscopically positive in 14 patients (20 %) and negative in 55 patients (80 %), including close margins (<1 mm) in 14 patients (20 %). Fifty-three patients (77 %) received radiotherapy. At a median follow-up of 41 months, there were 11 local (16 %) and 11 distant (16 %) recurrences. The local and distant 5-year recurrence-free survival rates were 72 % and 82 %, and the 5-year overall survival was 61 %. Increased age (scaled by 0.1; hazard ratio [HR] 1.80, P  = 0.002) and tumor size (HR 1.12, P  = 0.004) were negatively correlated with overall survival. Positive/close (<1 mm) margin status (HR 4.34, P  = 0.030) predicted worsened local recurrence-free survival. Conclusions MFS exhibit a propensity for local recurrence, which is predicted by resection with positive or close margins. Aggressive surgery combined with radiotherapy may contribute to more effective local control.

Echocardiography revealed a left atrial tumor in a 59-year-old man with back pain that concurrently worsened with left foot drop and loss of the left ankle reflex soon after admission to our hospital. Magnetic resonance imaging of the spine revealed an epidural tumor extending from Th5 with spinal cord compression. The patient was immediately treated by emergency Th4–5 laminectomy and epidural decompression. One month later, a cardiac tumor excised via the left atrial approach was histopathologically diagnosed as myxosarcoma, and the Th5 tumor was consistent with this finding. This is the first report to describe spinal metastasis of cardiac myxosarcoma.

A 36-year-old man suffering from exertional dyspnea was admitted to our hospital. An echocardiogram showed a left atrial tumor, which was excised via the left atrial approach. Histological examination of the surgical specimen revealed that it was a myxosarcoma. Twelve months after the surgery, the tumor recurred in the left atrium, and metastatic tumors were found in the right atrium and right ventricle. Surgery was performed once again, but examinations 5 months after the second surgery showed local recurrences and multiple metastases. Although the patient had received chemotherapy, he died 30 months after the first operation.

An 18- to 25-yr-old intact female Bengal tiger (Panthera tigris) was diagnosed antemortem and postmortem with nasopharyngeal myxosarcoma metastatic to the lung, chronic lymphoplasmacytic otitis media, and lymphoplasmacytic and eosinophilic stomatitis. Myxosarcomas are rare in domestic animals and seldom metastasize; this tumor has not been previously reported in an exotic felid. Computed tomography of the skull was used during the diagnoses.

Objective The objective of this case report is to present a rare case of soft tissue myxofibrosarcoma in the right lower limb, emphasizing the diagnostic delay, therapeutic decisions, and clinical outcome over a 24-year course. Background Myxofibrosarcoma is a rare and aggressive soft tissue sarcoma that often affects older adults. It may be misdiagnosed due to its histological resemblance to benign tumors such as schwannomas and is associated with high local recurrence and delayed distant metastasis. Case presentation A 47-year-old mestizo male patient presented with a progressively enlarging, firm mass in the right thigh, initially associated with paresthesias in the right lower limb. He underwent surgical resection, with initial histological diagnosis of benign schwannoma. Adjuvant radiotherapy and brachytherapy were administered, and 14 years later, he developed local symptoms again. After 20 years, histopathology confirmed high-grade myxofibrosarcoma. Despite further surgery, chemotherapy, and radiotherapy, he developed bilateral pulmonary metastases. Amputation and palliative therapy followed, and he died at age 50 years due to metastatic complications. Conclusion This case illustrates the diagnostic and therapeutic challenges of MFS. It highlights the importance of early pathological confirmation, long-term surveillance, and the value of integrated, multidisciplinary treatment. Molecular profiling may aid future therapeutic decisions in advanced stages.

Primary intracranial myxofibrosarcoma is exceedingly rare, with less than 10 cases published. We present a case of a 23-year-old man with previous history of a primary low grade myxofibrosarcoma of the left parietal-occipital convexity resected in March 1999. He subsequently underwent several interventions for multiple local recurrent disease until March 2004. At that time, complete remission was documented. About 8 years later, in February 2012, the patient was admitted to the emergency room with refractory acute pulmonary oedema. On work up, sustained monomorphic ventricular tachycardia and hyperechoic myocardial mass with invasion of the right ventricular cavity were detected. Electrical cardioversion was unsuccessful and irreversible cardiac arrest followed. The autopsy confirmed multiple bilateral lung metastases, malignant pulmonary embolism and myocardial invasion by the primary tumour, with intracavitary cardiac thrombosis and absence of intracranial disease. To the best of our knowledge, this is the first report of extracranial metastases of this neoplasm.