Kaposi sarcoma of major salivary gland origin - A clinicopathologic seriesof six cases

BACKGROUND. Kaposi sarcoma (KS), one of the defining tumors of acquired imm une deficiency syndrome (AIDS), is rarely identified in the major salivary glands. To the authors' knowledge, no previous published series has evaluat ed the clinicopathologic aspects of this tumor in major salivary glands. METHODS. Six cases of salivary gland KS, diagnosed between 1970 and 1998, w ere retrieved from the files of the Oral and Maxillofacial Pathology Regist ry of the Armed Forces Institute of Pathology. Histologic features were rev iewed and special stains, immunohistochemical studies, and in situ hybridiz ation were performed (n = 4). Patient follow-up data were obtained. RESULTS. The patients included 6 men ages 20-73 years (average, 53.0 years) . Patients presented clinically with a mass in the submandibular (n = 4) or parotid (n = 2) gland region. Symptoms were present for a mean of 13.7 mon ths. The tumors measured 1-4 cm (average, 2.5 cm) in greatest dimension. Hi stologically, the tumors exhibited the usual features of KS: a spindle cell vascular proliferation arranged in fasciculated bundles, variable nuclear pleomorphism, mitotic figures, extravasated erythrocytes, and hyaline globu les. Five patients were serologically positive for human immunodeficiency v irus (HIV) (three homosexual males, one infected by a contaminated blood tr ansfusion, and one with an unknown risk factor). Human herpesvirus-8 (HHV-8 ) was present in all cases tested (n = 4). Patients were treated with surgi cal excision (n = 6), followed by chemotherapy (n = 1) for the single patie nt with other foci of KS (rectal). Three patients died of AIDS-related infe ctious complications and one of congestive heart failure, whereas the remai ning patients are alive with AIDS but free of salivary gland KS. CONCLUSIONS. Salivary gland enlargement is frequently identified in HIV pos itive or AIDS patients. Although rare, it is important to consider RS in th e differential diagnosis of other AIDS-related salivary gland manifestation s (infections and tumors). Cancer 2000;88:15-23. (C) 2000 American Cancer S ociety.