KS, Kaposi's sarcoma, AIDS, acquired immune deficiency syndrome, HAART, highly active antiretroviral therapy
A 50-year-old, asymptomatic, homosexual man was evaluated 4 years ago for pulmonary nodules. He was diagnosed with human immunodeficiency virus infection in 1990. He was treated for Pneumocystis carinii pneumonia and cryptococcal meningitis in 1994. His antiretroviral medications included lamivudine and saquinavir. He denied intravenous drug use and tobacco abuse. He did admit to occasional cocaine use. Physical exam revealed dark-brown macular skin lesions on his face and forearms. His CD4 count was 35 cells/mm3 and his human immunodeficiency virus ribonucleic acid load was 17,500 copies/mL. Computed tomography of the chest (Fig. 1A) showed bilateral lung nodules. Flexible bronchoscopy revealed dark-brown discrete endobronchial lesions (Fig. 2). Bronchoalveolar lavage was nondiagnostic. Analysis of the bronchoalveolar lavage for human herpes virus-8 deoxyribonucleic acid was not performed. Video-assisted thoracoscopic lung biopsy demonstrated slit-like vascular lesions with atypical spindle-shaped cells and extravasated red blood cells consistent with Kaposi's sarcoma (KS) (Fig. 3). Biopsy of the skin lesions demonstrated chronic dermatitis. Antiretroviral therapy was changed to stavudine, lamivudine, and indinavir. He did not receive any radiation or chemotherapy. Subsequently, he had regression of the disease. A computed tomographic scan of the chest performed 2 years later showed resolution of the pulmonary nodules (Fig. 1B). There was a concomitant increase in CD4 to 440 cells/mm3 and a decrease in viral load to less than 50 copies/mL. Currently the patient is doing well and continues to have an active life-style.
KS is a slowly progressive angioproliferative tumor that had been reported to occur more commonly in patients with acquired immune deficiency syndrome (AIDS). 1 KS is an AIDS-defining illness (by the Centers for Disease Control) and is the most common tumor in AIDS patients. 2 In the early 1980s, 40% of homosexual men with AIDS presented with KS lesions. This number decreased dramatically to 10 to 20% in early 1990s. 3 The etiology of KS is still unknown, but a link between KS and human herpes virus-8 has been reported. 4 The most common site of involvement is the skin. Other organs that may be involved include the oral cavity, gastrointestinal tract, lymph nodes, spleen, liver, and respiratory system. 1,5 Pulmonary KS without skin involvement is rare. Combining two studies collectively, 38 of 39 AIDS patients with pulmonary KS also had cutaneous disease. 6,7 Our patient had endobronchial and parenchymal KS without any skin disease.
Clinical features of pulmonary KS are nonspecific. One series of 123 AIDS patients with pulmonary KS, and no concomitant pneumonia, reported cough in 92%, dyspnea in 82%, fever in 67%, chills in 44%, chest pain in 33%, and hemoptysis in 29% of the patients. 8 Our patient was asymptomatic. Sometimes it is difficult to differentiate pulmonary KS from pneumonia because of similar radiographic and clinical presentations. There is no physical examination finding specific for pulmonary KS. Chest radiographic findings may have a variety of patterns including normal, parenchymal and mediastinal abnormalities, bronchial thickening, and pleural effusion. 5–8 The most common findings on computed tomographic scans of the chest in AIDS-related pulmonary KS are ill-defined nodules. Other findings on chest computed tomography include perihilar infiltrates extending into the pulmonary parenchyma along bronchovascular bundles, interlobular septal thickening, fissural nodularities, discrete areas of ground-glass attenuation, pleural effusions, and hilar–mediastinal lymphadenopathy. 9 Sequential thallium and gallium scans had been reported to be useful in diagnosing pulmonary KS. Lee et al. 10 demonstrated a negative gallium scan and a positive thallium scan as being a typical pattern seen in KS lesions. Pulmonary KS is the most common endobronchial lesion associated with human immunodeficiency virus. 1 Bronchoscopy should be performed to diagnose endobronchial KS and to exclude infectious or noninfectious etiologies. Visualizing a typical tracheobronchial KS lesion is adequate for the diagnosis. A typical KS lesion is an erythematous or violaceous macule or papule. 8 Biopsy of a suspected tracheobronchial KS lesion is usually nondiagnostic because of the submucosal location of the tumor.
In addition, clinically notable hemorrhage has been reported to occur after biopsy in as many as 30% of the patients in certain series. 11 Garay et al. 12 reported a diagnostic yield of transbronchial biopsy to be 12% (2 of 17 patients). Transbronchial needle aspiration is also not diagnostic because the amount of cytology may not be adequate to make a diagnosis. Bronchoalveolar lavage may be helpful because the detection of human herpes virus-8 in bronchoalveolar lavage has been shown to be highly sensitive and specific for tracheobronchial KS. 4 Open lung biopsy was reported to have a diagnostic yield of 56%. 12 A lung biopsy may not always be necessary if the clinical and endobronchial manifestations are consistent with KS and/or the patient has advanced disease and is unable to undergo invasive procedures. Our patient had typical bronchoscopic findings; however, video-assisted thoracoscopic biopsy was performed to exclude a secondary process. Histologically, KS lesions contain spindle-shaped stromal cells with slitlike spaces filled with red cells, intertwined with vascular channels. 13
The prognosis of patients with pulmonary KS without treatment is poor, with a median survival of 2 to 10 months. 11,14 Chemotherapy can improve symptoms temporarily, but has a modest improvement in survival. Cadranel et al. 15 treated 30 AIDS patients with symptomatic pulmonary KS with adriamycin, bleomycin, and vincristine, and reported improvement of dyspnea but a median survival of only 6.5 months. Radiation therapy has also been studied. Meyer 16 treated 25 AIDS patients with symptomatic pulmonary KS with whole-lung radiation. There was symptomatic improvement, but the survival rate was only 15.7 weeks. More recently, highly active antiretroviral therapy (HAART) consisting of two nucleoside analogs and a protease inhibitor has been shown to increase survival. A case report by Aboulafia 17 described regression of an AIDS-related pulmonary KS with HAART. Two series of 15 patients with pulmonary KS who were treated with HAART reported nine complete remissions, three partial remissions, and three with progressive disease. 18,19 The median survival was more than 12 months. Our patient had a remarkable response to HAART, with resolution of pulmonary nodules, increase in CD4 count, and decrease in viral load.
Our patient demonstrates several unusual points. This patient had AIDS-related tracheobronchial KS without skin involvement, and this case underscores the bronchoscopic findings that may be present with endobronchial KS. However, a lung biopsy was necessary in our patient despite typical bronchoscopic KS lesions to exclude infectious or noninfectious etiologies. He had a remarkable clinical response and improvement of radiographic abnormalities with HAART.
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