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Left atrial myxosarcoma with systemic metastasis: a case report.

M S Roh1, G Y Huh, J S Jeong

  • 1Department of Pathology, Dong-A University College of Medicine, Pusan, Korea.

Journal of Korean Medical Science
|April 6, 2001
PubMed
Summary

This case report details a rare cardiac myxosarcoma in a young male, presenting with metastasis. The patient achieved long-term remission after multi-modal treatment, highlighting a potential therapeutic approach for this aggressive tumor.

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Area of Science:

  • Oncology
  • Cardiology
  • Pathology

Background:

  • Myxosarcoma is not a standard soft tissue tumor classification, typically reserved for cardiac tumors.
  • Primary cardiac myxosarcoma is exceptionally rare and challenging to distinguish from cardiac myxoma.
  • This report focuses on a unique case of left atrial myxosarcoma with extensive systemic metastasis.

Observation:

  • A 21-year-old male presented with dyspnea and orthopnea due to a large left atrial tumor.
  • Echocardiography revealed a mobile, pedunculated 7.5x5x2 cm mass in the left atrium.
  • Histopathology showed features resembling cardiac myxoma but with focal areas of high cellularity, nuclear pleomorphism, and frequent mitoses, indicative of malignancy.

Findings:

  • The patient underwent combination chemotherapy, stem cell transplantation, and surgical resection of systemic metastases.

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  • The tumor exhibited widespread metastasis to the brain, skeletal muscle, and lungs.
  • Despite the aggressive nature and metastasis, the patient remained alive and disease-free at 37 months post-diagnosis.

    • Implications:

    • This case highlights the importance of considering myxosarcoma in the differential diagnosis of cardiac tumors, even with myxoma-like features.
    • Aggressive multimodal therapy can achieve long-term remission in patients with primary cardiac myxosarcoma and systemic metastasis.
    • Further research is warranted to understand the biology and optimize treatment strategies for this rare entity.