Extraskeletal myxoid chondrosarcoma of the nasal cavity

Ceylan, Kürşat; Kizilkaya, Z.; Yavanoğlu, Ahmet

doi:10.1007/s00405-006-0102-2

Cited by 15 publications

(19 citation statements)

References 6 publications

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“…Extraskeletal myxoid chondrosarcoma is an extremely rare clinical entity characterized by chondroid and neurogenic differentiation in extraskeletal locations 1 . The incidence of ems is less than 2.5% among all soft-tissue sarcomas.…”

Section: Discussionmentioning

confidence: 99%

“…Extraskeletal myxoid chondrosarcomas traditionally behave less aggressively than do other more common forms of chondrosarcomas; as such, they are deemed "low grade." Their histologic characteristics often lead to a misdiagnosis as the myxoid variant of another sarcoma such as liposarcoma, mesenchymal chondrosarcoma, or malignant fibrous histiocytoma, or as parachordoma or another myxoid soft-tissue tumour 1,8 . However, detailed pathology analyses have shown that ems is a distinct clinical entity characterized by cytogenetic and molecular aberrations.…”

Section: Discussionmentioning

confidence: 99%

“…However, cases have occasionally been reported in children and adolescents. On average, men are more commonly affected than women 1 . Approximately 66% of these tumours originate in the lower extremities and limb girdles, followed by the distal extremities (23%) and trunk (13%) 9 .…”

Section: Current Oncology-volume 17 Numbermentioning

confidence: 99%

“…Diameter at presentation ranges from 6 cm to 13 cm, with a typical multinodular or nodular configuration, a poorly defined fibrous capsule, and well-defined margins 9 . Because of their predilection for hemorrhage, these tumours may be mistaken for hematomas 1 .…”

Section: Current Oncology-volume 17 Numbermentioning

confidence: 99%

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Extraskeletal Myxoid Chondrosarcoma of the Heart and Review of Current Literature

Geyer

Karlin

2010

Current Oncology

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echocardiogram showed no evidence of a cardiac structural abnormality, thrombus, or mass. The patient was placed on anti-platelet medication without further workup.In September 2007, the patient returned with newonset left flank pain, intermittent back pain, and visual disturbances. He also described the development of a mass in his left chest wall which had been increasing in size over the year. Physical exam confirmed the presence of a chest wall mass, and a harsh grade 3/6 systolic murmur was heard over the pulmonary valve on cardiac examination. Computed tomography (ct)-guided biopsy revealed a 2.0×3.5-cm low-to intermediate-grade myxoid sarcoma. The neoplastic cells were negative for wide-spectrum cytokeratin, S-100 protein, desmin, and smooth muscle actin.Transesophageal echo revealed a large (2.4×6.3-cm) mass of the right ventricular outflow tract, prolapsing into the pulmonary artery, with attachment at the crista supraventricularis; a right ventricular systolic pressure of 65 mmHg; and a 1.7×1-cm mass on the right aortic valve cusp resulting in severe aortic regurgitation. Gadolinium-enhanced mri of the brain demonstrated 5 intracranial masses within the right temporal lobe, the left occipital lobe, the right thalamus, the left frontal lobe, and the right parietal cortex.On combined positron-emission tomography/ct (pet-ct) staging, diffuse uptake was seen in the lateral aspect of the left 5th and 6th ribs, consistent with the patient's known tumour. Intense focal uptake was also evident within the right gluteus maximus, and faint activity was observed adjacent to the right second rib and the proximal jejunum. A focal cold defect was identified within the right atrium, extending into the main pulmonary artery.The patient was referred to cardiothoracic surgery for immediate removal of the right ventricular mass and excision of the native aortic valve. Pathology results from cardiac specimens confirmed a myxoid sarcoma, consistent with extraskeletal myxoid chondrosarcoma (ems). A biopsy of the right mesial temporal region confirmed metastatic disease. The patient was referred to oncology for further treatment. ABSTRACTExtraskeletal myxoid chondrosarcoma (ems) is a rare oncologic phenomenon characterized by chondroid and neurogenic differentiation in extraskeletal locations. These tumours represent fewer than 2.5% of all soft-tissue sarcomas and are most commonly found in the lower extremities, limb girdles, distal extremities, and trunk. Their presence in cardiac tissue is exceedingly unusual; just a single case of ems metastatic to the heart has been reported, and no cases of primary cardiac ems are known.Here, we report the case of a 26-year-old man who presented to his physician with a chest wall mass. Further evaluation led to the discovery of a large intracardiac mass with multiple end-organ growths. Complete work-up of this patient included cardiac biopsy, echocardiography, magnetic resonance imaging, positron-emission tomography, computed tomography, and fluorescence in situ hybridization studies fo...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Current Oncology-volume 17 Numbermentioning

confidence: 99%

Section: Current Oncology-volume 17 Numbermentioning

confidence: 99%

See 2 more Smart Citations

Extraskeletal Myxoid Chondrosarcoma of the Heart and Review of Current Literature

Geyer

Karlin

2010

Current Oncology

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“…1,2 There are a very few reports of extraskeletal myxoid chondrosarcoma in the head and neck. 5 On clinical examination, extraskeletal myxoid chondrosarcoma has no specific findings. In most cases, the neoplasm occurred as a single slow-growing, soft-tissue swelling, usually measuring 5-10 cm in diameter.…”

Section: Discussionmentioning

confidence: 99%