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Primary cardiac endosarcoma: a case report

  • Open Access
  • 26-09-2025
  • Sarcoma
  • Case report
Published in:

Abstract

Background

Primary cardiac intimal sarcoma is an exceptionally rare and aggressive malignancy, representing a small subset of primary cardiac tumors. Owing to its rapid progression and limited treatment options, the median survival is typically less than 1 year. We report a rare case of primary cardiac intimal sarcoma with MDM2 amplification, in which the patient achieved survival exceeding 1 year through a multimodal treatment approach, offering valuable insights into the management of this highly lethal disease.

Case presentation

A 48-year-old previously healthy Han Chinese woman presented with progressive chest tightness, dyspnea, and hemoptysis. Imaging revealed a large, irregular mass (64 × 41 mm) in the left atrium, partially prolapsing into the left ventricle. Elevated serum tumor markers were noted. The patient underwent surgical resection under cardiopulmonary bypass, and histopathological examination confirmed intimal sarcoma with MDM2 gene amplification. The diagnosis is: primary cardiac endosarcoma. Despite postoperative recurrence within the left ventricle, sequential therapies were implemented, including liposomal doxorubicin, oral anlotinib, and combined chemotherapy with gemcitabine and docetaxel plus anlotinib. The tumor initially showed progression but subsequently demonstrated partial regression following therapy adjustments. The patient has remained clinically stable for over 1 year post-diagnosis under ongoing treatment.

Conclusion

This case highlights the extreme rarity and poor prognosis of primary cardiac intimal sarcoma and demonstrates that combining surgery, chemotherapy, and targeted therapy may contribute to prolonged survival. These findings suggest the potential role of anlotinib-based therapy for managing MDM2-amplified cardiac intimal sarcoma and warrant further clinical investigation.
Title
Primary cardiac endosarcoma: a case report
Authors
Shishi Zhong
Yanhong Luo
Yunxia Fei
Siyu Zhang
Publication date
26-09-2025
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2025
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-025-05538-y
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Image Credits
Echocardiography of patient with primary cardiac endosarcoma/© 2025, Shishi Zhong et al, J Med Case Reports, Cancer cell screening and treatment concept/© artacet / iStock / Getty Images Plus, Colon cancer illustration/© (M) KATERYNA KON/SCIENCE PHOTO LIBRARY / Getty Images