TN 16 (03-18)
DI 23022.655 Pulmonary Kaposi Sarcoma
COMPASSIONATE ALLOWANCE INFORMATION
PULMONARY KAPOSI SARCOMA
Pulmonary KS; PKS
Kaposi Sarcoma (KS) is a cancerous tumor of the connective tissue involving blood and lymphatic vessels. KS is often associated with AIDS (Acquired Immune Deficiency Syndrome). When KS occurs in the lungs, it is referred to as Pulmonary Kaposi Sarcoma (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 Kaposi sarcoma 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 CODING
Chest CT and 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.
ONSET AND 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.
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:
Documentation of HIV infection
Clinical description of findings, imaging, pathology report of biopsied tissue, and laboratory studies
Suggested Listings for Evaluation:
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.