Myxofibrosarcoma or myxoma: malignant transformation or misdiagnosis

Inadequate excision, seeding, multicentricity and malignancy are often ascribed for recurrence of atrial myxomas. Differentiation between benign myxomas and malignant myxofibrosarcomas can be challenging, and a high index of clinical suspicion and vimentin staining on immunohistochemistry is needed to confirm the diagnosis. Early recurrence is common in these patients and completeness of excision is the key. Prognosis despite successful excision is poor. We report a benign atrial myxoma in a young female patient which upon recurrence turned out to be a myxofibrosarcoma.