Key result
Resection and chemotherapy for cardiac malignant fibrous histiocytoma yield ~13 months survival before fatal recurrence.
Case Report (n=1)
No
This case report highlights a rare presentation of primary cardiac malignant fibrous histiocytoma with vulvar metastases, demonstrating an aggressive clinical course and poor prognosis.
Vulvar metastasis signals aggressive cardiac MFH; extends sparse case literature but leaves management questions open.
Malignant fibrous histiocytoma (MFH) occurring in the heart is rare. To the best of our knowledge, the current study was the first to report a case of left atrial MFH metastasizing to the vulva. A 37-year-old female presenting with a history of exertional dyspnea underwent echocardiography, which revealed a mass in the left atrium, with a short-wide stalk attached to the posterior wall, extending into the pericardium. Furthermore, the mass resulted in severe mitral stenosis during diastole. The tumor was excised together with a section of the left atrial wall. The initial diagnosis was a left atrial myxoma. However, post-operative frozen section histopathology results indicated a diagnosis of MFH. Following surgery, the patient received chemotherapy, however, vulvar metastases and left pleural effusions were identified after 1 month. Following surgical excision of the vulvar tumor and left thoracentesis, the patient began adjuvant chemotherapy. However, the patient succumbed due to local recurrence 6 months later.
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Sun et al. (2015) conducted a case report in Primary cardiac malignant fibrous histiocytoma with vulvar metastases (n=1). Surgical resection and chemotherapy was evaluated on Clinical outcome / Survival. Following surgical resection and chemotherapy for primary cardiac malignant fibrous histiocytoma, the patient developed vulvar metastases and succumbed to local recurrence 13 months after diagnosis.
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