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Background Recurrence or metastasis of myxomas is not rare and can lead to malignancy.We aimed to analyze the risk factors for postoperative cardiac myxoma recurrence and to summarize its clinical characteristics,treatments and classification.Methods The clinical data of 5 patients with recurrent cardiac myxoma were retrospectively analyzed and our clinical experience was summarized.Moreover,the relevant literatures were reviewed.Results All the five cases of primary myxomas were derived from atypical positions.One patient had early distant metastasis,one had family history,and two suffered malignant recurrence.The recurrence interval was (2.30±2.16) years and the recurrent tumors were all found in different chambers from those of the corresponding primary tumors.Re-operation was performed after recurrence.One patient died of heart failure after malignant recurrence,and the other 4 cases had satisfactory therapeutic outcomes after re-operations.Our experience advocated a clinical classification of "typical" and "atypical" cardiac myxoma,the typical myxomas referred to the tumors locating at the left atria,with single pedicle,rooted at or around the fossa ovalis,involving no genetic causes,and the atypical myxomas included the familial tumors,tumors stemming from multiple chambers,rooted in abnormal positions of the left atrium,with evident genetic mutation,or with malignant tendency.Conclusions Postoperative follow-up is of vital importance for patients with myxomas characterized by multi-chamber distribution,early distant metastasis,atypical origin,and family history.Once recurs,re-operation is necessary and should be performed immediately.