A cardiac malignant primary tumor: Left atrium myxosarcoma-A case report.

Myxosarcoma only accounts for a very small proportion of primary malignant tumors of the heart. The disease has no specificity in clinical manifestations or features on medical images. In this case study, we report about a middle-aged female patient afflicted with cardiac myxosarcoma. The initial transthoracic echocardiography of the patient revealed a cardiac myxoma. However, the postoperative histopathology confirmed the presence of a malignant cardiac myxosarcoma. Post-surgery follow-up imaging examinations revealed local recurrence in the left atrium as well as soft-tissue and bone metastases. The recurrent tumor and metastases were subsequently treated with chemotherapy and radiotherapy. However, the tumor did not respond to treatment and the disease progressed.

Aggressive myxoinflammatory fibroblastic sarcoma with multiple site metastases.

Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare soft tissue sarcoma which was initially observed in acral sites and characterised by spindle cells, pleomorphic bizarre cells and distinctive large Reed-Sternberg-like cells admixed with an intense inflammatory cell infiltrates. MIFS manifests as a slow growing often superficial lesion which can be mistaken as infectious or chronic inflammatory process or benign tumours such as nodular fasciitis, giant cell tumour of tendon sheath or synovial pseudocyst. We report a rare presentation of a MIFS in a 38-year-old man with extensive local spread from subcutaneous tissue to the ankle joint and bones as well as multiple synchronous metastases to lung, sixth rib and vertebra. Our case is peculiar for its aggressive clinical behaviour with short duration, fast growth and extensive metastases, a feature infrequent in MIFS.

Myxofibrosarcoma of the extremity and trunk: a multidisciplinary approach leads to good local rates of LOCAL control.

AIMS: Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities. Locally infiltrative growth means that the risk of local recurrence is high. We reviewed our experience to make recommendations about resection strategies and the role of the multidisciplinary team in the management of these tumours. PATIENTS AND METHODS: Patients with a primary or recurrent MFS who were treated surgically in our unit between 1997 and 2012 were included in the study. Clinical records and imaging were reviewed. A total of 50 patients with a median age of 68.4 years (interquartile range 61.6 to 81.8) were included. There were 35 men; 49 underwent surgery in our unit. RESULTS: The lower limb was the most common site (32/50, 64%). The mean size of the tumours was 8.95 cm (1.5 to 27.0); 26 (52%) were French Fédération Nationale des Centres de Lutte Contre le Cancer grade III. A total of 21 (43%) had positive margins after the initial excision; 11 underwent further excision. Histology showed microscopic spread of up to 29 mm beyond macroscopic tumour. Local recurrence occurred in seven patients (14%) at a mean of 21 months (3 to 33) and 15 (30%) developed metastases at a mean of 17 months (3 to 30) post-operatively. CONCLUSION: High rates of positive margins and the need for further excision makes this tumour particularly suited to management by multidisciplinary surgical teams. Microscopic tumour can be present up to 29 mm from the macroscopic tumour in fascially-based tumours. Cite this article: Bone Joint J 2016;98-B:1682-8.

Cardiac myxosarcoma with thoracic spinal metastasis.

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.

Prognostic factors and outcomes of patients with myxofibrosarcoma.

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.

Surgical treatment 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.

[Ethical discussion of palliative sedation]. / Ethische Uberlegungen zur palliativen Sedierung.

This article discusses ethical implications of palliative sedation at the end of life. Terminal or palliative sedation, like other denominations are discussed in literature, which invite to a profound discussion. From an ethical point of view, terminal or palliative sedation seems to be a way out when in intolerable suffering or situations without prospects, the demand for euthanasia arises. The possibility to use palliative sedation for effective control of otherwise refractory suffering provokes the question if it even can be used with the intention to hasten death. This has led to ethical concerns on the use and limits of sedation. Therefore it seems to be necessary to elaborate clear guidelines in handling sedation in end-of-life care to prevent misuse. The contribution of ethical considerations can clarify the position and use of palliative sedation.

[Palliative sedation therapy for severe dyspnoea]. / Palliative Sedierung zur Symptomkontrolle massiver Dyspnoe.

In this case study we report on a patient with advanced myxofibrosarcoma and lung metastases, suffering from severe dyspnoea. Symptoms were not manageable by means of standard palliative care, which made palliative sedation therapy necessary in order to alleviate dyspnoea. On the basis of this case study, indications, drugs, therapy monitoring and ethical aspects of palliative sedation therapy are discussed.

Myxosarcoma in the eye and brain in a dog.

A male mixed breed dog of unknown age was presented with sudden onset of blindness and a slightly exophthalmic right eye. Ophthalmoscopically, a spherical, pinkish mass protruding from the region of the optic disc into the vitreous was seen in the right eye. Ultrasound and computer tomography demonstrated an extension of the mass into the right retrobulbar space, continuing intracranially to the optic chiasm. Cytologic findings, obtained by ultrasound-guided fine needle aspiration from the retrobulbar space, were consistent with myxosarcoma. On the basis of these findings of a well-delineated mass in the brain stem area, irradiation was planned as the therapy of choice. The dog was treated under general anesthesia using a proton beam at the Paul Scherrer Institute (PSI, Villigen, Switzerland). A curative protocol (56 Gy in 16 fractions over 4 weeks, 3.5 Gy/fraction) was used. Twenty-seven days post radiation therapy the dog was euthanized, as clinical symptoms progressed. Enlargement of the intracranial tumor was seen on a CT study repeated on the day of euthanasia. A gross pathologic and histologic examination were performed and confirmed the diagnosis of a myxosarcoma involving the eye, retrobulbar space of the right side, and the optic chiasm. Post-mortem examination failed to identify any abnormalities or metastases elsewhere in the body.

Extraskeletal myxoid chondrosarcoma of the thoracic wall.

The authors report the CT features of a very rare case of Extraskeletal Myxoid Chondrosarcoma of the thoracic wall, one of a few malignant primary bone tumors which rarely originate from soft tissues.

Nasopharyngeal myxosarcoma in a Bengal tiger (Panthera tigris).

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.

Left atrial myxosarcoma with systemic metastasis: a case report.

The term myxosarcoma is currently not used in standard classification for soft tissue tumors, but restricted to cardiac tumors. Primary cardiac myxosarcoma is a very rare disease and is difficult to differentiate from myxoma clinically and pathologically. We report a case of left atrial myxosarcoma with widespread systemic metastasis in a 21-yr-old male. The patient presented with sudden onset of intermittent dyspnea and orthopnea. Echocardiography showed a mobile, pedunculated tumor, 7.5x5x2 cm in size, at left atrium. Histologically, the excised tumor showed an amorphous finely fibrillar and mucinous stroma, in which irregular cords and clusters of lepidic cells and large stellate cells with plump vesicular nuclei resembled the usual type of cardiac myxoma were noted. And it showed focally cellular area with great nuclear pleomorphism and frequent mitoses. The patient received combination chemotherapy, peripheral blood stem cell collection transplantation and operations for systemic metastases in the brain, skeletal muscle and lung. He is alive at present 37 months after initial diagnosis and has no more new metastatic lesion.