Classic Kaposi's sarcoma associated with human herpesvirus 8 infection in a 13-year-old male : A case report

• Published in: Clinical cancer research 2001-08, Vol.7 (8), p.2263-2268
• Main Authors: LANDAU, Heather J, POIESZ, Bernard J, DUBE, Samalima, BOGART, Jeffrey A, WEINER, Leonard B, SOUID, Abdul-Kader
• Format: Article
• Language: English
• Subjects: Adolescent; Antiviral Agents - therapeutic use; Base Sequence; Biological and medical sciences; Dermatology; DNA, Viral - genetics; Herpesvirus 8, Human - drug effects; Herpesvirus 8, Human - genetics; Humans; Immunoglobulins, Intravenous - therapeutic use; Interferon-alpha - therapeutic use; Male; Medical sciences; Molecular Sequence Data; Polymerase Chain Reaction; Sarcoma, Kaposi - drug therapy; Sarcoma, Kaposi - pathology; Sarcoma, Kaposi - virology; Sequence Homology, Nucleic Acid; Skin - drug effects; Skin - pathology; Skin Neoplasms - drug therapy; Skin Neoplasms - pathology; Skin Neoplasms - virology; Tumors of the skin and soft tissue. Premalignant lesions
• ISSN: 1078-0432; 1557-3265

Classic Kaposi's sarcoma (KS) is rare in children. Although its etiology is not fully understood, human herpesvirus 8 (HHV-8) is present in the angiogenic lesions. We report an HIV-negative, 13-year-old patient of Sicilian descent with HHV-8-associated classic KS to facilitate the diagnosis and treatment of this entity in children. DNA was extracted from the skin specimen of the patient and analyzed via PCR assay and Southern blot hybridization for HHV-8 DNA. The amplified HHV-8 DNA was cloned, sequenced, and compared with the prototype HHV-8-KS330/BAM. The patient presented with purpuric lesions on the distal lower extremities and the tip of his nose, associated with thrombocytopenia and leukopenia, suggesting an immune-mediated cytopenia. While on prednisone, he developed marked vascular proliferation in the groins. Biopsy of the skin lesions showed KS, and HHV-8 was detected in the tissues by PCR. Sequence analysis of the amplified DNA was homologous to the prototype HHV-8-KS330/BAM. His HHV-8 strain was the A subgroup, the type associated with Mediterranean classic KS. Stopping prednisone and treatment with IFN-alpha and IgG resulted in regression of the groin lesions. This report emphasizes the importance of recognizing classic KS in children and avoiding immunosuppressive therapies in indolent classic KS. The diagnostic and therapeutic strategies were effective and well tolerated.