We report a case of sporadic giant cardiac myxoma with a rare localization in the right atrium, operated in our Service, in a 73-year-old female patient who also presented a patent foramen ovale and a history of ischemic stroke in the year prior to current admission. Intra-operatively, the tumor had a very friable, gelatinous aspect, with a high potential for embolization due to its reduced consistency. The present paper refers to clinical, histochemical and immunohistochemical particularities, as well as to macroscopic and microscopic characteristics of the cardiac myxoma, emphasizing the extracellular matrix aspects, and without leaving out the cellular components of this rare tumor, with possible inference in the management of this disease. The authors present their own observations related to the data from the literature. Also, there are some particularities of the case which justify the current presentation.

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