Pulmonary Kaposi Sarcoma: A Cryptic Angiosarcomatous Malignancy With Fatal Outcome

Abstract

SESSION TITLE: Lung Cancer 1 SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Monday, October 30, 2017 at 03:15 PM - 04:15 PM INTRODUCTION: Pulmonary kaposi sarcoma (KS) is a rare neoplasm in HIV patients due to Human Herpes Virus-8 (HHV-8). Lack of specific symptomatology impede a high clinical suspicion leading to a delay in initiation of appropriate therapy. CASE PRESENTATION: A 22-year-old female with congenital HIV, off combined antiretroviral therapy (cART), presented with stabbing chest pain, chronic cough and dyspnea. On exam, she had rhonchi, distended abdomen, splenomegaly and diffuse lymphadenopathy. No oral or skin lesions. Laboratory tests showed cytopenias and acute kidney injury. CD4 131/9%. Quantiferon Gold negative. CT angiogram demonstrated diffuse interstitial infiltrates, hepatic lesions, ascites and mediastinal and inguinal lymphadenopathy. She underwent inguinal lymph node excisional biopsy which was consistent with metastatic KS. cART were promptly initiated. Bronchoscopy was performed to assess for hemoptysis. It revealed hyperemic lesions throughout the tracheobronchial tree. On day 11 of cART, patient developed respiratory failure requiring intubation. Despite starting steroids, she rapidly declined and developed multiorgan dysfunction before expiring on comfort measures. DISCUSSION: Although KS incidence is the highest among malignancies in AIDS patients, diagnosis is delayed as 70% of patients lack cutaneous manifestations. Median survival was 2 to 6 months before advent of cART. Early initiation of chemotherapy is delayed but recommended in pulmonary KS, even without significant change in survival time. While endobronchial biopsies are discouraged, visualization of pathognomic lesions, or BAL for HHV-8 PCR may prove beneficial. Deleterious outcomes including death are common. Although rarely reported, KS-related inflammatory response immune system (IRIS) can occur as early as second week of cART. Chemotherapy combined with cART can control the symptoms of IRIS and resolve KS. Our patient had a rapid clinical deterioration with fatal outcome before chemotherapy could be initiated. CONCLUSIONS: Pulmonary KS warrants a high clinical suspicion and prompt initiation of chemotherapy. Although rarely encountered by pulmonologists, recognition of the characteristic lesions on bronchoscopic visualization is mandated in order to make an accurate diagnosis. In this era of potent cART, careful monitoring for KS-related IRIS after starting cART is crucial. Despite combination of cART and chemotherapy, the prognosis is reported dismal. Reference #1: Connick E et al. Clin Infect Dis. 2004 Reference #2: Garay SM et al. Chest. 1987 Reference #3: Odongo FC. Case Rep Infect Dis. 2013 DISCLOSURE: The following authors have nothing to disclose: Zahia Esber, Sarah Barbour, Jason Stansberry No Product/Research Disclosure Information