1344330 - A Rare Case of Metastatic Pleomorphic Sarcoma with Left Atrial Infiltration

Description of Clinical Presentation: A 61-year-old male with known, pathology-confirmed left facial pleomorphic dermal sarcoma treated with wide excision two years prior presented with a chief complaint of hemoptysis for three days. He had been experiencing non-specific left-sided chest pain worse with inspiration for one month. He denied fever, chills, congestion, recent illness, or recent sick exposures. He also denied active or prior tobacco use.

Diagnostic Techniques and Their Most Important Findings:

Computed tomography (CT) of the chest with contrast for further investigation showed an 8.4 cm lingular mass with a tumor thrombus invading into the left superior pulmonary vein and left atrium (Figure 1). Lung biopsy confirmed the presence of metastatic pleomorphic sarcoma. Subsequent transthoracic echocardiogram (TTE) showed a 6.5 cm × 2.2 cm left atrial mass crossing the mitral valve annulus (Figure 2) and whole-body position emission tomography-CT (PET-CT) scan with fluorodeoxyglucose (FDG) showed maximum standardized uptake value (SUVmax) 18.7 of the left atrial mass and SUVmax 25.4 of the left lower lung lobe mass (Figure 3).

Due to his persistent hemoptysis and pathology-confirmed diagnosis of metastatic pleomorphic sarcoma, chemotherapy with gemcitabine/docetaxel and palliative radiation were initiated. Serial imaging was done with TTEs and PET-CTs, as some cases have shown cardiac metastases being missed on chest CTs. Findings showed a gradual decrease in size of the atrial mass as well as improvement in upper respiratory symptoms.

Learning Points from this Case:

Metastatic cardiac sarcomas are extremely rare neoplasms with varying clinical progression. They are associated with a poor prognosis overall and can cause heart failure, arrythmias, and malignant pericardial effusions.¹ Diagnosis should include multi-modality imaging with TTE, CT, PET-CT, and cardiac magnetic resonance (CMR) imaging.² Although there are no uniform guidelines on treatment strategies, chemotherapy and palliative radiation may be pursued to slow disease and symptom progression.^2,3 Surgical resection can be challenging depending on infiltration, size, and anatomy of the tumor, but is sometimes used for palliative reasons.²