Key result
A young man initially misdiagnosed with a cardiac myxoma experienced local recurrence at 3 months, leading to a revised diagnosis of cardiac intimal sarcoma and subsequent adrenal metastasis at 1 year.
Why the study?
Cardiac intimal sarcomas are extremely rare and aggressive primary malignant cardiac tumors with risks of misdiagnosis, uncertain potential for malignant transformation of myxomas, and challenging therapeutic modalities.
Case Report (n=1)
This case highlights the risk of misdiagnosing extremely rare and aggressive cardiac intimal sarcomas as benign cardiac myxomas, emphasizing the need for careful histopathological analysis and follow-up.
Alerts to sarcoma risk in recurrent cardiac masses; leaves open standardized diagnostic protocols.
Cardiac intimal sarcoma are extremely rare and aggressive primary malignant cardiac tumors. Here, we reported the case of a young man initially operated for a tumor of the left atrium, causing a dynamic obstruction of the mitral valve and (mis-)diagnosed as a myxoma at the histopathological analysis. Patient presented a local recurrence at 3 months and was reoperated. Pathology revealed this time the presence of an intimal sarcoma. Patient received adjuvant chemotherapy. Despite a good local control, the one-year follow-up PET scan revealed the presence of a metastasis in the left adrenal gland that was surgically resected. This paper aims to highlight the risk of misdiagnosis in case of cardiac tumors, the hypothetical concept of malignant transformation of a cardiac myxoma, the aggressive course of the extremely rare cardiac intimal sarcoma and the therapeutic modalities available to treat this pathology.
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Durieux et al. (2020) conducted a case report in Cardiac intimal sarcoma (n=1). Surgical resection and adjuvant chemotherapy was evaluated. A young man initially misdiagnosed with a cardiac myxoma experienced local recurrence at 3 months, leading to a revised diagnosis of cardiac intimal sarcoma and subsequent adrenal metastasis at 1 year.
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