An Interesting Differential for Orthopnoea: Leiomyosarcomas

 Abstract

Background

Primary cardiac tumors are very rare. The autopsy incidence is between 0.0001-0.030%. 25% of the tumors are malignant and 95% of them represent primary cardiac sarcomas. Primary Cardiac Sarcomas (PCS) can be histologically divided into angiosarcomas, leiomyosarcoma and fibrosarcoma. This case report describes a rare case of leiomyosarcoma that presented late with metastasis.

Case Summary

Mrs. H, 67, presented with an 18-month history of dry cough, and 3 months of worsening breathlessness and orthopnoea. Chest X-ray (CXR) and Computed Tomography (CT) chest findings prompted an urgent transthoracic echocardiogram which showed a large sessile mass filling in the right atrium with some obstruction to the mitral valve (MV) and appeared attached to the posterior MV annulus. Under Cardiology, patient had a CT Chest-Abdomen-Pelvis (CT CAP) with contrast which showed an obstructing lesion involving the left atrium, infiltrating through the MV into the left ventricle and disseminated bony metastasis. A core biopsy of L3 paravertebral mass showed moderately differentiated leiomyosarcoma. The patient had Magnetic Resonance Imaging (MRI) of the spine after reporting back pain which showed metastasis to the vertebral bodies of T3, T10, T12 and L3 (posteriorly eroded and infiltrated by soft tissue mass). A multi-disciplinary team discussion yielded that this patient receives palliative radiotherapy to L3 vertebrae.

Conclusion                              

PCS should be considered as a differential when patients present with worsening cardiovascular symptoms, albeit rare. This case report adds to the existing literature of primary cardiac sarcomas and advocates for primary care services to have open access for requesting echocardiograms for early diagnosis of such tumours.

Keywords

Orthopnoea; Primary Cardiac Sarcomas; oncology; echocardiogram; pericardial effusion; leiomyosarcomas