An uncommon long-term survival case of primary cardiac leiomyosarcoma
© Glaoui et al.; licensee BioMed Central Ltd. 2014
Received: 20 January 2013
Accepted: 20 October 2014
Published: 10 November 2014
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.
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.
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 paravertebral soft tissue mass was revealed by magnetic resonance imaging. There was no lung metastasis. The patient received antalgic vertebral radiotherapy with systemic palliative chemotherapy consisting of oral cyclophosphamide. One year later, he died of progressive disease and multiorgan failure.
Primary leiomyosarcomas of the heart are extremely rare, accounting for 0.019% of all malignant cardiac neoplasms in autopsy studies. They typically occur in the fourth decade with no sex predilection. As all other cardiac sarcomas, primary leiomyosarcoma of the heart are usually asymptomatic until an advanced stage. Consequently, the diagnosis is often clinically delayed until hemodynamic consequences and embolisms. Cardiac MRI is the gold standard complementary exam to distinguish malignant lesions and assess the resectability of the tumors.
Cases of cardiac leiomyosarcoma reported in the literature with available data of treatment modalities and survival
Curative surgery and adjuvant chemotherapy
Curative surgery and adjuvant chemotherapy
Curative surgery and adjuvant radiotherapy
Surgery and palliative chemotherapy
Oral informed consent was obtained from the patient for publication of this case report and any accompanying images.
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