Case 2 -

Kaposi's Sarcoma Joanne Lynne Wright, University of British Columbia, Vancouver, BC, Canada

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Case History 59 year old male HIV positive for 8 years: HAART therapy Rectal B cell lymphoma previous year treated conservatively Cutaneous Kaposi's with eye involvement Previous admission 20 days prior to death for management Presents 5 days prior to death with: productive cough shortness of breath fatigue Physical Exam Multiple cutaneous Kaposi's sarcoma lesions Bulky lymph nodes in bilateral inguinal and left posterior cervical areas Inspiratory crepitations in left mid lung and right lung base Radiology Admission Chest X-Ray: Infiltrates in both lower lobes and right middle lobe with subsegmental atelectasis and right basilar pleural effusion Marked septal thickening. CT: One week prior to admission: Peribronchial soft tissue infiltration with bronchial narrowing. Focal nodular infiltrates. Bilateral small pleural effusion. Three days after admission: Marked progression of disease with accentuation of lymphatics, increased nodularity, consolidation, and increase in size of pleural effusions.. Case 2 - Figure 1 Gross specimen of lung Case 2 - Figure 2 Whole mount of selected slide Case 2 - Figure 3 High power magnification Clinical Differential Diagnosis Infection (PCP, MAI, bacterial) Pulmonary malignancy Kaposi Sarcoma lymphoma Immune reconstitution syndrome Cryptogenic organizing pneumonia Pathologic Diagnosis : Kaposi's sarcoma Differential Diagnosis of Pulmonary Infiltrates in a Patient with HIV Disease and Anti-retroviral Therapy a) Infections: Community acquired bacterial pneumonia Immunodeficiency related pneumonias Pneumocystis Mycobacterial ( MAC, M-TB) Fungal (dimorphic / mycelial) Toxoplasmosis Viral (herpes) b) Disease related to HAART therapy: Immunoreconstitution inflammatory syndrome Drug reaction c) Malignancies: Kaposi's sarcoma Lymphoma Epithelial malignancies Kaposi's Sarcoma 1. Classical Kaposi's sarcoma Primarily skin related May involve viscera in 10% as a late phenomenon Found in patients of Eastern Europe and Mediterranean origin, marked male predominance 2. Endemic Kaposi's sarcoma African, HIV negative More common in children and adolescents Epidemiology complicated by irregular HIV testing data 3. Immunosuppression / transplant related Kaposi's sarcoma 0.1 to 1.0 % of transplant recipients May resolve with cessation of immunosupression 4. AIDS associated Almost all cases of Kaposi's are HHV8 positive [can be helpful as a diagnostic tool] Virus encodes proteins homologous to oncoproteins Bcl-2 like anti-apoptotic protein Protein cyclin related to cell growth Inhibitor of interferon signaling pathway Encodes chemokines which activate angiogenesis and inhibit Th1 T-cell response Presentation of Kaposi's sarcoma in the lung cough in more than 50% of patients fever in 20% dyspnea in approximately 10% pain / hemoptysis / lymphadenopathy less than 5% each Radiological Differential Diagnosis of Kaposi's Sarcoma by Pattern Focal Consolidation More common in non-PCP pneumonia Reticular Infiltrates Classical for PCP pneumonia BUT Found in almost same frequency in non-PCP pneumonia Septal lines Feature of KS, lymphoma Nodular infiltrates Infections (MAC, TB) Lymphoma More advanced KS Micronodular infiltrates KS PCP, TB Ground glass opacities PCP Pleural effusions KS Non-PCP pneumonia Position of infiltrates Perihilar Symmetric – PCP Non summetric – KS Basal Non-PCP pneumonia Lymphadenopathy MAC Lymphoma HAART effects Developed KS Differential Diagnosis Related to CD4 count Less than 200 – PCP, MAC, Lymphoma, KS, non-PCP pneumonia More than 500 – bronchopneumonia Pathological Features of Kaposi's sarcoma Gross: discrete dark red and hemorrhagic nodules Gross and micro: follows lymphatic distribution (septa and bronchovascular bundles) (useful diagnostic clue) Micro: spindle cells admixed with inflammatory cells (lymphocytes and plasma cells) in no specific cellular arrangement Cleft like spaces with intact or degenerate RBC Cytological features of cells Elongate nuclei Mild cellular atypia Occasional mitoses Cytoplasm with hyaline globules (degenerate RBC) Immunohistochemistry HHV 8 positive CD 34, CD31 positive D2-40 positive VEGFR-3 positive Factor VIII negative Differential Diagnosis of Vascular Proliferations in the Lung Benign: hemangioma / lymphangioma Patient population : any age Gross: May be well demarcated or ill defined, but in no specific place in the lung Micro: cavernous or capillary spaces Endothelial cells bland No mitotic activity Immunohistochemical profile Both CD 31 positive Hemangioma CD 34 positive Lymphangioma D2-40 positive Malignant: Hemangioendothelioma Patient population 50% less than 40 years of age Often asymptomatic High ratio females:males Radiology: multiple nodules with or without ground glass opacities Gross: nodules grey in color with "chondroid" texture Microscopic: nodules associated with small vessels and lymphatics, but with intraalveolar and interstitial growth Greater intensity of cellularity along periphery of nodules Cytology: cells round to spindled Intracytoplasmic lumena with may have RBC Stroma chondroid or hyaline Immunohistochemistry Factor VIII, CD 31, CD 34 positive 50% CD 7 positive Malignant: Angiosarcoma (non-great vessel disease) Patient population: wide age range, male:female 3:1 Symptoms: chest pain, cough, hemoptysis / hemothorax Radiology: interstitial / airspace infiltrates / masses Diffuse / single / multiple Gross: lymphatic distribution Microscopic: irregular anastomosing vascular channels Ranging from cavernous to capillary to slid like Cytology: atypical endothelial cells with "hobnail" appearance Cellular crowding forming papillary projections and solid nodules Pleomorphic cells with high mitotic rate Intracytoplasmic vacuoles and microlumena Immunohistochemistry Factor VIII, CD 31 CD 34 positive References Schlossbauer et al: Pulmonary radiological characteristics in patients with HIV infection at the time of highly active antiretroviral therapy (HAART). Eur J Med Res 2007; 12:341 Weissferdt et al: Primary vascular tumors of the lungs: a review. Ann Diag Pathol 2010:14 296 Antman et al: Kaposi's sarcoma. New England Journal of Medicine 2000; 343: 1027 Allen et al: Imaging lung manifestations of HIV/AIDS. Annals of Thoracic Medicine 2010; 5:201 Kandemir et al: Lymphatic differentiation in Classic Kaposi's sarcoma: Patterns of D2-40 immunoexpression in the course of tumor progression. Pathology Oncology Research 2010 DOI 10.1007/s12253-011-9392-9