Differential Diagnosis and Surgical Management of Cardiac Sarcomas in Children and Young Adults: State of the Art and Case Report

I. Hüners (Hamburg)1, M. Munz (Hamburg)1, G. K. Lund (Hamburg)2, T. Giertsch (Hamburg)3, H. Carstens (Hamburg)1, J. Sachweh (Hamburg)1, P. M. Clemmensen (Hamburg)4, L. E. M. Hannen (Hamburg)5, D. Biermann (Hamburg)1, S. Blankenberg (Hamburg)4, A. Duprée (Hamburg)6, R. Kozlik-Feldmann (Hamburg)1, M. Hübler (Hamburg)1, C. Rickers (Hamburg)7
1Universitätsklinikum Hamburg-Eppendorf Klinik für Kinderherz­medizin und EMAH Hamburg, Deutschland; 2Universitätsklinikum Hamburg Eppendorf Klinik und Poliklinik für Diagnostische und Interventionelle Radiologie und Nuklearmedizin Hamburg, Deutschland; 3Universitätsklinikum Hamburg-Eppendorf Klinik für Radiologie Hamburg, Deutschland; 4Universitäres Herz- und Gefäßzentrum Hamburg Klinik für Kardiologie Hamburg, Deutschland; 5Universitäres Herz- und Gefäßzentrum Hamburg Allgemeine und Interventionelle Kardiologie Hamburg, Deutschland; 6Universitätsklinikum Hamburg-Eppendorf (UKE) Allgemein-, Viszeral- und Thoraxchirurgie Hamburg, Deutschland; 7Kinderherzmedizin und EMAH, Universitätsklinikum Hamburg-Eppendorf Bereich Erwachsene mit angeborenem Herzfehler Hamburg, Deutschland

Introduction

A sarcoma of the heart, medically referred to as a cardiac sarcoma, is a malignant tumor that develops within the heart’s structures. Magnetic resonance imaging (MRI) is one of the preferred diagnostic tools for such tumors, as it provides detailed soft-tissue imaging. Cardiac angiosarcomas, the most common malignant subtype, often present with nonspecific symptoms, delaying diagnosis. Typical MRI features include isointense to slightly hyperintense signals on T1-weighted images, hyperintense signals on T2-weighted images, and heterogeneous gadolinium enhancement.

 

Case Report and Methods

A 34-year-old female presented with exertional dyspnea, fatigue, and chest pressure, initially suspected as recurrent perimyocarditis. Echocardiography showed RA wall thickening and pericardial effusion. MRI revealed a semi-circumferential right atrial mass with heterogeneous gadolinium enhancement, isointense T1, and hyperintense T2 signals, reflecting necrosis and edema, according to Table 1. Furthermore, PET-CT confirmed a metabolically active lesion without metastasis. Radical tumor resection was performed, involving RA, interatrial septum, superior vena cava, and partial left atrium resection, followed by reconstruction with a xenopericardial patch and pacemaker implantation.

 

Results

Intraoperative findings confirmed an invasive tumor extending from the RA to adjacent structures. The tumor was carefully resected in the healthy tissue, and the right atrium was reconstructed using a xenopericardial patch. Postoperative MRI showed complete resection. Histopathology revealed a high-grade angiosarcoma with necrosis. Postoperative challenges included transient cardiac decompensation and symptomatic bradyarrhythmia, resolved with diuretics and pacemaker implantation. The patient commenced adjuvant chemotherapy with paclitaxel and radiotherapy alongside fertility preservation measures.

 

Conclusion

This case underscores the pivotal role of multidisciplinary management in treating cardiac sarcomas. Before surgery, MRI and PET-CT are essential for differential diagnosis and assessment of the tumor´s extension and metastasis. Advanced cardiovascular surgery with radical resection and right atrial reconstruction showed an excellent functional and histological result. Additional oncological therapy and thorough follow-ups with MRI are recommended.