Abstract 17945: Characterization and Treatment of Primary Cardiac Rhabdomyosarcoma in the Last Three Decades

Introduction: Cardiac rhabdomyosarcomas are rare, aggressive neoplasms comprising 5% of cardiac sarcomas overall. Due to the low prevalence, there is little consensus on the appropriate identification and treatment of this condition. This research aims to characterize trends in the clinical presentation of the disease and identify improvements for therapeutic management.

Methods: A literature review was conducted by identifying cases of primary cardiac rhabdomyosarcoma published in English to PubMed between 1993 and 2023 and available via the SUNY Downstate Library. Studies were analyzed for patient demographics, clinical course, treatment, and recovery.

Results: The analysis included 31 identified cases. Most cases were reported in male (52%) adults (87%) with an average age of 44 (STD: 21). Patients frequently presented with dyspnea (59%) and cardiac issues (52%), including chest pain (19%) and conduction disorders (19%). Tumors were detected via echocardiogram (79%) and CT (18%) and frequently left-sided (60%), with the most cases arising in the left atrium (47%). There was valvular involvement in 43% of cases. Surgery was performed in 86% of patients, of which 33% were fully resected via sternotomy and 13% were cardiac transplants for unresectable tumors. 56% of patients survived at 12 months follow-up. Surgical management was combined with chemotherapy (46%), such as the VAC regimen (40%), and radiation (14%), which improved patient 1-year mortality by 26%. 26% of patients developed recurrence of the primary tumor and 42% of patients developed metastatic disease; osseous metastases (38%) were the most common. 13% of patients had a prior history of cancer.

Conclusions: The rare rhabdomyosarcoma may present as a middle-aged patient with dyspnea and cardiac concerns refractive to standard management. A left atrial mass mimicking a benign lesion such as a myxoma may be seen on echocardiogram, and suspicious lesions should undergo surgical resection to relieve symptoms and confirm the diagnosis. Supplementation of surgical management using chemotherapy and radiation may improve outcomes in patients. Clinicians should closely monitor the patient for osseous and lung metastases. A prior history of cancer may be a risk factor for the disease.