Abstract
Extraskeletal 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 for the translocation involving the EWSR1 gene locus (22q12). Results of the foregoing studies confirmed the diagnosis of ems, but the origin of this patient's tumours remains elusive and the contention between a primary cardiac source and cardiac metastasis has yet to be resolved.This article describes the histopathology, immunohistochemistry, and chromosomal aberrations common to ems, together with the common presenting features, natural history, and prognosis.
Highlights
We report the case of a 26-year-old man who presented to his physician with a chest wall mass
A transesophageal echocardiogram showed no evidence of a cardiac structural abnormality, thrombus, or mass
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
Summary
In February 2007, a 26-year-old man presented to his primary care physician with a history of a 30-minute episode of light-headedness, vertigo, ataxia, and left-sided extremity numbness. In September 2007, the patient returned with newonset left flank pain, intermittent back pain, and visual disturbances He described the development of a mass in his left chest wall which had been increasing in size over the year. The patient had been employed as an electronics technician, and he denied tobacco or excessive alcohol exposure His family history included a grandmother with kidney cancer. The patient was subsequently switched to gemcitabine for 1 cycle, but he experienced rapid progression of his disease, predominantly within the abdominal cavity. He was given doxorubicin, but had no response. The patient died 17 months from the time of diagnosis
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