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.

Acute Presentation of a High-Grade Myxofibrosarcoma Originating in the Thoracic Wall: A Case Report.

We report the first case of a patient with myxofibrosarcoma (MFS) who presented acutely with a rib fracture and developed a rapidly expanding loculated hemothorax after chest trauma. The patient was taken to the operating room for evacuation of hemothorax, and samples and biopsy specimens were taken for cytologic and pathologic examination. Final report with immunohistochemical staining showed a high-grade MFS. After the procedure, there was clinical and radiological improvement, and the patient was followed up as an outpatient. Myxofibrosarcoma is a very rare and aggressive connective tissue neoplasm with variable presentations. Surgical resection is the preferred treatment. Prompt diagnosis and adequate management of these tumors are important to reduce their high local recurrence and distant metastasis rates. Therefore, it is important to be aware of its common and uncommon presentations.

Radiomic features from MRI distinguish myxomas from myxofibrosarcomas.

BACKGROUND: Myxoid tumors pose diagnostic challenges for radiologists and pathologists. All myxoid tumors can be differentiated from each other using fluorescent in-situ hybridization (FISH) or immunohistochemical markers, except for myxomas and myxofibrosarcomas. Myxomas and myxofibrosarcomas are rare tumors. Myxomas are benign and histologically bland, whereas myxofibrosarcomas are malignant and histologically heterogenous. Because of the histological heterogeneity, low grade myxofibrosarcomas may be mistaken for myxomas on core needle biopsies. We evaluated the performance of T1-weighted signal intensity (T1SI), tumor volume, and radiomic features extracted from magnetic resonance imaging (MRI) to differentiate myxomas from myxofibrosarcomas. METHODS: The MRIs of 56 patients (29 with myxomas, 27 with myxofibrosarcomas) were analyzed. We extracted 89 radiomic features. Random forests based classifiers using the T1SI, volume features, and radiomic features were used to differentiate myxomas from myxofibrosarcomas. The classifiers were validated using a leave-one-out cross-validation. The performances of the classifiers were then compared. RESULTS: Myxomas had lower normalized T1SI than myxofibrosaromas (p = 0.006) and the AUC using the T1SI was 0.713. However, the classification model using radiomic features had an AUC of 0.885 (accuracy = 0.839, sensitivity = 0.852, specificity = 0.828), and outperformed the classification models using T1SI (AUC = 0.713) and tumor volume (AUC = 0.838). The classification model using radiomic features was significantly better than the classifier using T1SI values (p = 0.039). CONCLUSIONS: Myxofibrosarcomas are on average higher in T1-weighted signal intensity than myxomas. Myxofibrosarcomas are larger and have shape differences compared to myxomas. Radiomic features performed best for differentiating myxomas from myxofibrosarcomas compared to T1-weighted signal intensity and tumor volume features.

Upper extremity myxofibrosarcoma mimicking an erosive inflammatory arthritis: a case report.

Myxofibrosarcoma is a malignant fibroblastic soft tissue neoplasm containing a variable amount of myxoid stroma that commonly presents as a slow-growing mass in elderly patients. The neoplasm may be superficial or deep to the muscle fascia and characteristically has an infiltrative growth pattern with a dominant or multinodular mass. We describe an unusual case of high-grade myxofibrosarcoma of the wrist and forearm that infiltrated the muscles, tendons, and wrist joint, causing bone erosions. The tumor was mistakenly diagnosed as synovitis and a chronic, erosive, inflammatory process. The diffuse nature, absence of a dominant mass, and radiographic appearance complicated the diagnosis. Although neoplasms of the synovial spaces are rare, this case demonstrates that tumors with a highly infiltrative growth pattern can mimic inflammatory synovitis and that neoplasms should be considered in the differential diagnosis when clinical and laboratory features are discordant with the imaging appearance.

Extracardiac intrapericardial myxosarcoma causing right ventricular outflow tract obstruction in a dog.

A 13-year-old male castrated pomeranian cross was referred for evaluation of episodes of collapse and a suspected cardiac mass. The presence of a mass at the base of the heart within the pericardial space was confirmed by echocardiography. Additional diagnostics included computed tomography, ultrasound-guided fine-needle aspirate, and thoracic radiographs. The mass was surgically debulked and diagnosed as myxosarcoma via histopathology. This case report describes the diagnostic imaging, laboratory findings, and short-term positive clinical outcome of a dog with a myxosarcoma in a previously undescribed location.

Primary atrial fibromyxosarcoma with multiple-system metastases: A case report.

RATIONALE: Fibromyxosarcoma is common in head and neck, vessel, omentum, and reproductive system, with low-grade malignant behavior. However, primary atrial fibromyxosarcoma with highly malignant behavior is extremely rare. PATIENT CONCERNS: A 34-year-old female presented with oppression in the chest, short breath, and onset of headache as initial symptoms. The preoperative echocardiogram showed a medium-size echogenic mass close to the posterior leaflet of the mitral valve in the left atrium. DIAGNOSIS: Primary atrial fibromyxosarcoma with multiple-system metastases. INTERVENTIONS: The patient underwent surgery, and the tumor was removed completely. The diagnosis of left atrium fibromyxosarcoma was confirmed through postoperative histopathological examination. Positron emission tomography/computed tomography scan was performed, which revealed multiple metastases to left adnexa, bilateral adrenal glands, left iliacus, right lateral ventricle, and skeletal system. OUTCOMES: The patient died of cerebral hernia caused by hemorrhage from the metastatic brain tumor, 30 days after the surgery, without receiving chemotherapy or radiotherapy. LESSONS: Cardiac fibromyxosarcoma is a rare primary malignant cardiac neoplasm, probably with systemic metastases. The possibility of malignancy should be considered as differential diagnosis for cardiac mass.

[Myxofibrosarcoma in the abdominal cavity]. / Myxofibrosarkom dutiny brisní.

A number of benign and malignant tumors may develop in the abdominal cavity. Sarcomas are rather rare tumors of the abdominal cavity. They are often diagnosed at advanced growth stages as their local growth can cause clinical problems to the patients. The author presents a case report of myxofibrosarcoma in the abdominal cavity.Key words: myxofibrosarcoma.

Head and neck myxofibrosarcoma: a case report and review of the literature.

INTRODUCTION: Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, peaking in the seventh decade, and it is mainly encountered in the lower extremities. Myxofibrosarcoma of the head and neck are extremely rare. To the best of our knowledge, only 19 cases have been described in the head and neck so far. This is a literature review and retrospective chart review of our experience in head and neck myxofibrosarcoma treatment in our department. CASE PRESENTATION: In this case report we describe a 35-year-old Caucasian man who presented the first case of myxofibrosarcoma arising from the pterygopalatine fossa. The peculiar anatomical location and the extent in the midcheek region make this case a hard "challenge" for the surgeon, in order to guarantee wide surgical margins of resection. A total right maxillectomy was accomplished by means of the Weber-Ferguson approach, preserving the orbital floor. The excised portion was reconstructed using the free rectus abdominis myocutaneous flap. Postoperative radiotherapy was given to the area adjacent to the lesion, with a total dose of 60 Gy. No relapse occurred in the 27-month postoperative follow-up. CONCLUSIONS: The case described suggests the importance of combined surgical and adjuvant radiotherapy to avoid local and distant recurrences of the tumor. In our opinion, combined surgical and adjuvant radiotherapy followed by close clinical observation to search for a metastatic disease is advisable in all cases. Further studies are needed to confirm the efficacy of combined radio-chemotherapy for head and neck myxofibrosarcoma in terms of long-term disease-free survival.

Myxoinflammatory fibroblastic sarcoma: a case report.

Myxoinflammatory fibroblastic sarcoma (MIFS) is a recently described, rare low-grade sarcoma. Generally located in the upper and lower extremities, MIFS clinically mimics a benign cystic mass and is composed of spindle-like or atypical cells and mixed inflammatory infiltrates located in the fibroblastic myxoid stroma. Radiologic images and macroscopic appearance generally resemble a lobulated mass with irregular margins. We present a case of a tumoral mass with neoplastic cells at the center and a smooth surface with a previously undefined appearance. Myxoinflammatory fibroblastic sarcoma is significantly difficult to distinguish clinically from benign lesions and the surgeon should consider the possibility of malignancy in lesions located at the extremities.

F-18 FDG PET/CT imaging of bulky myxofibrosarcoma in chest wall.

Myxofibrosarcoma (MFS) has a spectrum of malignant fibroblastic lesions with variably myxoid stroma and pleomorphism. A 67-year-old man with a bulky mass on his chest wall was diagnosed with MFS. He underwent F-18 fluorodeoxyglucose (FDG) positron emission tomography/computed tomography for detection of metastasis. FDG positron emission tomography /computed tomography showed inhomogeneous high FDG uptake (max standardized uptake value, 10.1) in the bulky tumor with no evidence of metastasis, and the tumor was successfully resected. FDG uptake seemed to be reflected by the broad spectrum of pathologic heterogeneity. And MFS should be considered when making a diagnosis of inhomogeneous FDG-avid lesions in the bulky masses of soft tissue.

Imaging features of orbital myxosarcoma in dogs.

Myxomas and myxosarcomas are infiltrative connective tissue tumors of fibroblastic origin that can be distinguished by the presence of abundant mucinous stroma. This paper describes the clinical and imaging features of orbital myxosarcoma in five dogs and suggests a predilection for the orbit. The main clinical signs were slowly progressive exophthalmos with soft swelling of the pterygopalatine fossa, and in two dogs, of the periorbital area. No pain was associated with the eye or orbit but one dog had pain on opening the mouth. The dogs were imaged using combinations of ultrasonography, radiography, and magnetic resonance imaging. In four dogs, extensive fluid-filled cavities in the orbit and fascial planes were seen and in the fifth dog, the tumor appeared more solid with small, peripheral cystic areas. In all dogs, the lesion extended along fascial planes to involve the temporomandibular joint, with osteolysis demonstrable in two dogs. Fluid aspirated from the cystic areas was viscous and sticky, mimicking that from a salivary mucocoele. Myxomas and myxosarcomas are known to be infiltrative and not readily amenable to surgical removal but their clinical course seems to be slow, with a reasonable survival time with palliative treatment. In humans, a juxta-articular form is recognized in which a prominent feature is the presence of dilated, cyst-like spaces filled with mucinous material. It is postulated that orbital myxosarcoma in dogs may be similar to the juxta-articular form in man, and may arise from the temporomandibular joint.

Left atrium myxosarcoma: an exceptional cardiac malignant primary tumor.

We report the case of a 32-year-old patient with a left atrium myxosarcoma, presenting with congestive heart failure. It is a rare cardiac malignant primary tumor that seems to derive from the same cellular line as myxomas, but the prognosis is very different. These tumors present local recidives and distance metastasis, so the mean survival is about 1 year, independent of any therapeutical option.

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.

Low-grade fibromyxoid sarcoma.

Described is a low-grade fibromyxoid sarcoma (LGFMS) of the abdominal wall muscles in a 38-year-old black woman. There was no evidence of metastatic disease. A 5.2-kg LGFMS - the largest case ever reported - was resected. One year after surgery, the patient is alive without any sign of local recurrence or distant metastasis. Follow-up comprises abdominal and thoracic CT scans at 6-month intervals.

Right atrial myxosarcoma in a dog.

A case of right atrial myxosarcoma is described in a Staffordshire bull terrier with a history of weight loss, exercise intolerance and collapse. The diagnosis of an intracavitary cardiac tumour was made on echocardiography. The dog was euthanased. Secondary spread to the lungs and lymph nodes was present. Myxoma is one of the rare intracavitary cardiac tumours and this case is believed to be the 1st report of its more malignant form in the dog.

Primary cardiac malignancy presenting as left atrial myxoma. Clinical and surgical considerations.

Primary heart neoplasms occur in 0.002-0.3% of autopsies: 30% are myxomas and 20-30% are malignancies, almost always sarcomas. Cardiac metastases are 10 to 40 times more frequent than primary heart cancer. We describe a case of a left atrial sarcoma erroneously diagnosed as myxomas preoperatively. Standard surgical indication for resection of cardiac myxomas is based on echocardiography. Because of the severity of cardiac malignant lesions than can mimic atrial myxomas at echocardiography, through preoperative investigation should be accomplished, best by magnetic resonance imaging. In case of suspected malignancy, total body computed tomography should be performed to avoid unnecessary cardiac operations in case of disseminated cancer. To date the only good medium and long-term results in the therapeutic management of heart sarcomas have been achieved by transplantation: the probable explanation is that criteria of surgical radicality should be those followed for soft tissue tumors located elsewhere in the organism.