Key result
Emergency surgical resection successfully removed a rare co-occurring primary cardiac leiomyosarcoma in the right ventricular outflow tract and a benign tumor in the main pulmonary artery.
Why the study?
Primary cardiac leiomyosarcoma is rare, and its coexistence with a benign tumor is even more uncommon.
Case Report (n=1)
This case highlights the rare coexistence of a primary cardiac leiomyosarcoma and a benign pulmonary artery tumor, emphasizing the role of surgical resection and the potential for misdiagnosis as pulmonary embolism.
May raise suspicion for cardiac leiomyosarcoma in PE-like cases; leaves open standardized diagnostic and therapeutic approaches.
Leiomyosarcoma is frequently found in the retroperitoneum, mesentery, omentum, uterus, or subcutaneous tissue. However, primary cardiac leiomyosarcoma is rare and even more uncommon is its coexistence with a benign tumor. We report a case involving a 61-year-old female with a right ventricular outflow tract leiomyosarcoma in conjunction with a benign mass located in the main pulmonary artery. Echocardiography revealed a 2.5 × 2.2 cm isoechoic mass in the right ventricular outflow tract and a 3.8 × 1.8 cm irregular isoechoic mass in the main pulmonary artery. Computed tomography angiography (CTA) indicated a patchy filling defect in a similar location. Initially, pulmonary embolism was considered, however, the possibility of tumors could not be excluded. Given the high risk of mass embolization, we proceeded with emergency surgery. A large, irregular, solid mass was found attached to the wall of the main pulmonary artery, fortunately without involvement of the pulmonary valve. Exploration of the right ventricular outflow tract uncovered an additional solid, smooth, well-encapsulated mass. Immunohistochemical analysis of the right ventricular outflow tract mass confirmed the presence of tumor cells that were positive for Desmin and smooth muscle actin (SMA), while negative for S-100 and myoglobin, leading to a diagnosis of leiomyosarcoma. For the pulmonary mass, Immunohistochemistry revealed the proliferation of fibrous tissue, mucus degeneration, and calcification within the focal area. The imaging characteristics of cardiac leiomyosarcoma combined with benign pulmonary artery tumors may be misinterpreted as thrombosis, however, surgical resection remains a viable treatment option.
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Wu et al. (2025) conducted a case report in Cardiac leiomyosarcoma and benign pulmonary mass (n=1). Surgical resection was evaluated on Tumor resection and pathological diagnosis. Emergency surgical resection successfully removed a rare co-occurring primary cardiac leiomyosarcoma in the right ventricular outflow tract and a benign tumor in the main pulmonary artery.
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