Kaposi sarcoma (KS) is the most common malignancy associated with human immunodeficiency virus (HIV) infection. Its prevalence and aggressiveness have decreased since antiretroviral therapy has become widespread, especially in developed countries. The clinical presentation of KS varies from indolent skin lesions to extensive visceral disease. The lungs and the gastrointestinal tract are the most common sites of visceral disease, although exclusive airway disease is quite rare.1
Pulmonary KS has been diagnosed by flexible bronchoscopy in 8% to 14% of patients with acquired immunodeficiency syndrome and respiratory symptoms, in 6% to 32% of patients with acquired immunodeficiency syndrome who had cutaneous KS, and in 21% to 49% of HIV-infected persons with known mucocutaneous KS and respiratory symptoms.2,3 Pulmonary KS in the absence of mucocutaneous involvement, as in our case, is rare.2
A 24-year-old male patient presented to the emergency department with a 2-week history of worsening productive cough, occasional blood-tinged sputum production, shortness of breath, hoarseness, weight loss, and fatigue.
His history was significant for homosexual unprotected encounters, intravenous recreational drug abuse, and HIV infection diagnosed in January 2009. He was also known to have hepatitis B and C viral infections. He was noncompliant with his clinical follow-ups, leading to lack of antiretroviral treatment.
On physical examination, the patient had extensive oral candidiasis. There were no cutaneous lesions. The chest x-ray revealed a bilateral interstitial infiltrate. The blood analysis revealed increased levels of lactate dehydrogenase (1188 U/L; normal range, 240 to 480 U/L) and C-reactive protein (17.67 mg/dL; normal range, 0.01 to 0.5 mg/dL).
The patient was severely immunocompromised with 770 lymphocytes per microliter (normal range, 1500 to 3000 lymphocytes/μL) and 2 TCD4+ lymphocytes/microliter (normal range, 700 to 1100 lymphocytes/μL). He was hospitalized and placed on empirical treatments for community-acquired pneumonia and Pneumocystis jirovecci infection and on oral antifungals for candidiasis.
Thoracic computed tomographic scan revealed multiple small irregular nodules involving centrilobular areas associated along with ground-glass opacities more prominent in the lung apices. Next day, he developed dyspnea and stridor.
Flexible bronchoscopy revealed a vascular lesion involving the arytenoids and producing significant narrowing of the glottis chink (Fig. 1A). The scope could not be introduced through the stenotic area, and hence a rigid bronchoscopy was performed. Necrotic debris involving anterior and lateral tracheal walls was observed (Fig. 1B, C). The right vocal cord was paralyzed, whereas the left one had reduced mobility. Dilation of the glottis area and upper trachea was performed using an 8.5 mm diameter rigid bronchoscope. Biopsy of the endobronchial lesions revealed proliferation of fusiform atypical cells, diagnostic of laryngeal, and tracheal infiltration by KS.
The patient initiated antiretroviral therapy and chemotherapy with doxorubicin, completing 6 cycles.
After 8 months, the patient had no respiratory complaints and showed recovery in his immune status [T CD4+ 137 lymphocytes/μL (normal range, 700 to 1100 lymphocytes/μL), virus load by polymerase chain reaction HIV1 21 copies/mL; normal range, undetected].
Pulmonary KS may be difficult to differentiate from other infectious or neoplastic conditions, yet the distinction is essential for therapeutic and prognostic purposes.2 The extent of tracheobronchial KS ranges from isolated tracheal lesions to diffuse and/or extensive tracheobronchial involvement as it was in our case where endobronchial lesions produced airway narrowing. The bronchoscopic appearance of endobronchial KS is considered to be characteristic enough to suspect the diagnosis,4 especially when mucocutaneous involvement is present. In our case, although the patient had characteristic KS lesions at bronchoscopy, a histologic confirmation was preferred due to its rarity.
Pulmonary KS can involve the parenchyma, the airways, the pleura, and the intrathoracic lymph nodes. Violaceous or bright red lesions are found in some patients in the mucosa of the lower airways, especially at branching points, or, much less commonly involving the upper airways.
Head and neck involvement of KS is not unusual. However, laryngeal involvement as in our case is considered a rather infrequent manifestation with approximately 40 cases reported.5–7 When KS involves the larynx, airway compromise can be a serious threat; immediate treatment is necessary, particularly through a rigid bronchoscope for both diagnostic and therapeutic intentions.
Our case highlights rare involvement of upper airways with KS, its unusual presentation, and the need for therapeutic bronchoscopy.
1. Dorothy AW. Pulmonary involvement in HIV-associated Kaposi's sarcoma; UpToDate, May 2009 avaliable at www.uptodate.com
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3. Palmieri C, Dhillon T, Thirlwell C, et al. Pulmonary kaposi sarcoma in the era of highly active antiretroviral therapy HIV Med.. 2006;7:291
4. Gasparetto TD, Marchiori E. Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology Orphanet J Rare Dis. 2009;4:18
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6. Alkhuja S, Menkel R, Patel B, et al. Stridor and difficult airway in an AIDS patient AIDS Patient Care STDS.. 2001;15:293–295
7. Roy TM, Dow FT, Puthuff DL. Upper airway obstruction from AIDS-related Kaposi's sarcoma J Emerg Med.. 1991;9:23–25