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Extra-Medullary Plasmacytoma of the Heart – Symptoms, Treatment & Support

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Posted by Matthew O’Connor | Jan 31, 2024

What Is Extra-Medullary Plasmacytoma of the Heart?

Extra-medullary plasmacytoma of the heart is a rare type of cancer that originates outside of the bone marrow in plasma cells, a type of white blood cell that produces antibodies. While plasmacytomas more commonly occur in tissues such as the nasal sinuses or throat, their presence in the heart is extremely rare. This condition involves the abnormal proliferation of plasma cells in the cardiac tissue, leading to tumour formation.

Symptoms

The symptoms of extra-medullary plasmacytoma of the heart can vary greatly depending on the tumour’s size and location within the heart but may include:

  • Shortness of breath or difficulty breathing, especially during physical activity.
  • Chest pain or discomfort, which may be mistaken for angina.
  • Abnormal heart rhythms (arrhythmias).
  • Fatigue and weakness.
  • Swelling in the legs or ankles due to fluid accumulation.
  • Dizziness or fainting spells.

Disease Aetiology (Causes)

The exact cause of extra-medullary plasmacytoma, including those occurring in the heart, remains unknown. Factors that may influence the development of plasma cell disorders include genetic mutations, environmental exposures, and a history of autoimmune diseases, though none have been definitively linked to this condition.

Diagnosis

Diagnosing extra-medullary plasmacytoma of the heart involves a combination of clinical assessment and diagnostic tests, including:

  • Imaging studies such as echocardiography, MRI, or CT scans to visualize the heart and detect any masses.
  • Biopsy of the cardiac tissue, if accessible, to confirm the presence of abnormal plasma cells.
  • Blood tests to evaluate for abnormal proteins produced by plasma cells and other indicators of systemic involvement.

Treatment

Treatment for extra-medullary plasmacytoma of the heart typically focuses on the eradication of the plasma cell tumour and may include:

  • Radiation therapy, which is often the primary treatment modality given the localized nature of the tumour.
  • Surgery to remove the tumour, if it is deemed safe and feasible based on its location and size.
  • Chemotherapy, particularly if there is evidence of more widespread disease or if the tumouur does not respond to radiation.
  • Targeted therapy and immunotherapy, which are emerging as potential options based on the tumouur’s specific genetic and molecular characteristics.

What support can we give for the Extra-Medullary Plasmacytoma of the Heart?

Extra-Medullary Plasmacytoma of the Heart is a rare cancer, meaning it is not as well-known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. So, we provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as PExtra-Medullary Plasmacytoma of the Heart, we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!

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