ALTERNATE NAMES

Cardiac Angiosarcoma; Heart Sarcoma; Malignant Heart Tumor; Primary Cardiac Tumor

Cardiac sarcoma is a rare type of primary malignant (cancerous) tumor that occurs in the heart. Primary cardiac sarcoma are extremely rare malignant tumors that start in the heart. Primary cardiac sarcoma is often diagnosed late. It is known to proliferate rapidly and generally carries a poor prognosis despite aggressive treatment. Cardiac tumors can occur at any age. However, primary cardiac sarcomas are more common in middle-aged adults.

The most common type of primary cardiac sarcoma is angiosarcoma. Other types of primary cardiac sarcoma include leiomyosarcoma, malignant fibrous histiocytoma, myxoma, rhabdomyosarcoma, and synovial sarcoma.

NOTE: Angiosarcoma, leiomyosarcoma, and rhabdomyosarcoma are CAL conditions. Evaluate claims for these conditions using the guidance provided in their impairment summaries.

Diagnostic testing: A diagnosis of primary cardiac sarcoma is confirmed by:

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  Transthoracic echocardiogram (TTE);

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  Transesophageal echocardiogram (TEE);

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  Electrocardiogram (ECG/EKG);

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  Cardiac magnetic resonance imaging (MRI);

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  Computed tomography (CT) scan;

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  Chest x-ray;

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  Positron emission tomography (PET) scan;

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  Biopsy; and

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  Cardiac catheterization/angiography.

Physical findings: Signs and symptoms of primary cardiac sarcoma may include:

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  Obstruction of the right atrium;

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  Swelling of feet, legs, ankles, and/or abdomen;

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  Increased fluid around the heart;

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  Chest pain;

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  Shortness of breath;

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  Fatigue;

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  Palpitations;

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  Hemoptysis (coughing up blood);

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  Heart rhythm problems;

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  Upper facial congestion;

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  Fever;

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  Weight loss;

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  Night sweats;

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  Malaise (fatigue, tiredness);

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  Fingers that change color or turn blue when pressure is applied; and

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  Nail curvature with enlargement of fingers.

ICD-9: 164.1

ICD-10: C38.0

While most cardiac tumors are benign, primary cardiac sarcomas are aggressive with a higher risk of metastasis and shorter survival rates compared to other soft tissue sarcomas.

Once primary cardiac sarcoma has progressed to the point that symptoms begin to occur, it has often metastasized (spread) to other parts of the body, making the associated treatment challenging. The type of treatment for primary cardiac sarcoma depends largely on the location and size of the tumor as well as the extent of metastasis. Types of treatment for primary cardiac sarcoma may include heart transplantation, open heart surgery, or autotransplantation.

If metastasis has occurred, radiation therapy or chemotherapy may be given to help relieve symptoms or improve quality of life.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Results from cardiac imaging; and

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  Biopsy results.

4.09

13.04A or B

Meets listing 4.09 only if heart transplant surgery has been performed.