An uncommon long-term survival case of primary cardiac leiomyosarcoma
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CASE REPORT
WORLD JOURNAL OF SURGICAL ONCOLOGY
Open Access
An uncommon long-term survival case of primary cardiac leiomyosarcoma Meriam Glaoui1, Zineb Benbrahim2*, Rhizlane Belbaraka3, Sara Naciri4, Hassan Errihani4 and Axel Lescene2
Abstract Primary cardiac sarcoma is a rare aggressive entity. It constitutes the second most common type of primary cardiac neoplasms. Its management has largely been guided by small retrospective series with a median survival of 6 months. Here, we discuss a unique case of 8-year survival cardiac leiomyosarcoma managed by surgical and adjuvant therapy. Keywords: Cardiac neoplasm, Leiomyosarcoma, Survival
Background Primary cardiac sarcomas constitute a rare entity. At present, only a few hundred primary cardiac sarcomas have been reported, most of which are based on autopsy series. Despite this rarity, they are the second most common type of primary cardiac neoplasm and account for most of the malignant primary cardiac tumors. Primary cardiac sarcomas are aggressive tumors that generally do not produce symptoms until they are locally advanced. Their management has largely been guided by small retrospective series and non-cardiac sarcoma management principles. However, the prognosis of primary cardiac sarcomas in general remains poor according to the current medical literature. The median survival in patients with cardiac sarcomas has been reported to be about 6 months with a mean of 11 months. Here, we discuss a unique case of 8-year survival cardiac leiomyosarcoma managed by surgical and adjuvant therapy. Case presentation A 47-year-old man presented with acute pulmonary edema with severe progressive dyspnea and orthopnea. He had a 10 pack-year history of cigarette smoking, but no other medical or familial conditions. Chest radiograph showed a small right-sided pleural effusion. Electrocardiogram revealed sinus tachycardia without any conduction abnormalities. Transthoracic echocardiography * Correspondence: [email protected] 2 Gustave Roussy Institute, 114 Rue Édouard Vaillant, 94805 Villejuif, France Full list of author information is available at the end of the article
and cardiac magnetic resonance imaging (CRMI) showed a tumor (4 × 5 cm) occupying the side wall of the right atrium budding into the lumen (Figure 1). Through a median sternotomy, a radical excision of the neoplasm was performed; the atrial wall was reconstructed using a bovine pericardial patch. The post-operative course was unremarkable and the anatomical pathology analysis revealed fusiform cells with considerable nuclear pleomorphism and mitotic activity. The immunohistochemical study was positive to vimentin, desmin, actin, and HHF-35, and negative for PS-100, cytokeratin, and hormone receptors. The final diagnosis was cardiac leiomyosarcoma. Adjuvant chemotherapy with doxorubicin (60 mg/m2 day 1) and ifosfamide (3 g/m2 day 1 and 2) were realized and MRI after the four cycles did not show any sign of local or distant relapse. Seven years after the first operation, local recurrence with a metastatic para
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