Primary cardiac sarcoma: insights from two decades of multimodal management at LMU Munich

Burkhard-Meier, Anton ; Di Gioia, Dorit; Jurinovic, Vindi ; Hoberger, Michael; Güler, Sinan E. ; Völkl, Michael; Corradini, Stefanie ; Gaasch, Aurélie V.; Alig, Annabel H. S.; Knösel, Thomas ; Hagl, Christian; Schneider, Christian; Sienel, Wulf; Kunz, Wolfgang G.; Burkhard-Meier, Caspar; Bergwelt-Baildon, Michael von ; Lindner, Lars H. und Berclaz, Luc M. (2025): Primary cardiac sarcoma: insights from two decades of multimodal management at LMU Munich. In: Cardio-Oncology, Vol. 11, 58

Abstract

Background: Primary cardiac sarcomas (PCS) are rare, aggressive malignancies with poor prognosis and limited evidence guiding optimal management. We aimed to evaluate clinical and histopathological parameters in a single-center PCS cohort.

Methods: Thirty-three patients diagnosed with PCS between 2002 and 2024 were retrospectively reviewed. Clinical outcomes and prognostic factors were analyzed. Event-free survival (EFS) was defined as the time from initial diagnosis to the first occurrence of disease progression, recurrence, or death. Overall survival (OS) was calculated from initial diagnosis to death.

Results: The median age at diagnosis was 45 years, with angiosarcoma representing the most common histological subtype (n = 9, 27%). Most patients presented with localized or regional disease (n = 25, 75%), predominantly involving the right (n = 11, 33%) and left atrium (n = 8, 24%). In patients who underwent comprehensive genomic profiling, MDM2 amplification was the most common molecular alteration (n = 5, 45%). The majority of patients received multimodal treatment: surgical resection in 76% (n = 25), systemic therapy in 73% (n = 24), and radiotherapy in 21% (n = 7). After a median follow-up of 63.4 months, median EFS and OS were 11.7 months (95% CI 9.4–23.7) and 37.5 months (95% CI 21.2–83.2), respectively. Distant metastasis (p = 0.027, HR = 3.74) and angiosarcoma histology (p = 0.014, HR = 6.97) were significantly associated with worse OS, while surgical resection was associated with improved OS (p = 0.0064, HR = 0.086).

Conclusion: Our findings underscore the key clinical and histopathological characteristics of PCS and suggest that surgical resection - even when incomplete - may confer a survival benefit in this aggressive tumor entity. The favorable clinical outcomes observed in this cohort may be attributable to the high proportion of patients undergoing multimodal treatment.

• Item Type: Journal article
• Keywords: Heart sarcoma; Multimodal treatment; Primary cardiac sarcoma; Soft tissue sarcoma
• Faculties: Medicine > Institute of Pathology; Medicine > Institute for Medical Information Processing, Biometry and Epidemiology; Medicine > Medical Center of the University of Munich > Clinic for General, Visceral, Vascular and Transplant Surgery; Medicine > Medical Center of the University of Munich > Cardiac Surgery Clinic and Polyclinic; Medicine > Medical Center of the University of Munich > Medical Clinic and Outpatient Clinic III (Oncology); Medicine > Medical Center of the University of Munich > Clinic and Polyclinic for Radiology
• Subjects: 600 Technology > 610 Medicine and health
• ISSN: 2057-3804
• Language: English