ALTERNATE NAMES

PKS; Pulmonary KS

DESCRIPTION

Kaposi sarcoma (KS) is a cancerous tumor of the connective tissue involving blood and lymphatic vessels. KS is often associated with AIDS (acquired immunodeficiency syndrome). When KS occurs in the lungs, it is referred to as pulmonary Kaposi sarcoma (pulmonary KS) or Kaposi’s sarcoma with pulmonary involvement. Pulmonary KS grows as sheets of tumor tissue in the peribronchial and perivascular interstitial spaces. Symptoms and signs of pulmonary KS include dyspnea (difficulty breathing), fever, non-productive cough, and hemoptysis (coughing up blood). Complications of pulmonary KS include respiratory failure caused by airway obstruction, parenchymal involvement, pleural effusion, or pulmonary edema from lymphangitic obstruction.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: Chest computed tomography (CT) and magnetic resonance imaging (MRI) may show findings suggestive of pulmonary KS, but the definitive diagnosis is obtained by bronchoscopy showing characteristic lesions. Transbronchial biopsy or open lung biopsy may be necessary if findings on bronchoscopy are inconclusive.

Physical findings: Symptoms and signs of pulmonary KS include:

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  Dyspnea (difficulty breathing);

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  Fever;

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  Non-productive cough;

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  Hemoptysis (coughing up blood);

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  Respiratory failure caused by airway obstruction;

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  Parenchymal involvement;

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  Pleural effusion; or

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  Pulmonary edema from lymphangitic obstruction.

ICD-9: 176.4

ICD-10: C46.50

PROGRESSION

Survival for pulmonary KS is variable (between 4 to 19 months) after it is first diagnosed. Related deaths are usually due to upper airway obstruction or parenchymal destruction.

TREATMENT

Chemotherapy in combination with antiretroviral therapy is used to treat pulmonary KS. Radiation therapy has also been used in cases where there is intolerance to chemotherapy. Radiation is also used to treat symptomatic airway obstruction.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Documentation of HIV infection; and

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  Clinical description of findings, imaging, pathology report of biopsied tissue, and laboratory studies.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

14.11

114.11

Equals