Benign and Malignant Smooth Muscle Tumors Containing Epstein-Barr Virus in Children with AIDS

Abstract

Smooth muscle tumors (leiomyosarcomas) are the second most prevalent malignancy of children with the acquired immunodeficiency syndrome (AIDS). We have investigated the tumors, plasma, and peripheral white blood cells of eight children with AIDS with smooth muscle tumors for evidence of tumor association with human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV). Very low levels of HIV were found in the tumors of the AIDS patients, probably resulting from blood-borne carriage of virus. These smooth muscle tumors had very high quantities of EBV in all the tumor cells by in situ hybridization, with an average of 4.5 EBV genomes per cell by quantitative polymerase chain reaction amplification. Increased amounts of EBV were found in the peripheral blood cells of two AIDS patients before the time of tumor diagnosis. EBV clonality studies demonstrated different monoclonal EBV infection of two separate colonic tumors from one patient, and dual or mixed monoclonal EBV infection in another patient. The muscle cells of leiomyomas and leiomyosarcomas of patients with AIDS demonstrated prominent staining with antibodies to the EBV receptor. The uniform distribution and striking amount of EBV in the tumor cells demonstrates that EBV is capable of infecting smooth muscle cells and that these cells support EBV replication. Clonal EBV proliferation suggests that EBV infection occurs at an early stage of tumor development. These findings indicate that EBV has a causal role in the oncogenesis of leiomyosarcomas of patients with AIDS.

Multifocal Epstein-Barr Virus-Associated Smooth Muscle Tumor in Adults with AIDS: Case Report and Review of the Literature

“…However, despite the degree of differentiation, the majority of EBV-SMT would be classified as sarcoma according to guidelines [17]. Conversely multifocality of tumor, suggesting multiple metastasis, could not be used to retain the diagnosis of malignity since a number of studies have shown that they correspond to the presence of multiple independent tumors from different clones [8, 10, 11, 14]. Necrosis is also possible.…”

“…It is noteworthy that analysis of clonality of these different tumors, assessed by molecular analysis of the long terminal repeat region of EBV genome, showed the presence of different clones in tumors from different sites [8, 10, 11, 14] suggesting that they represent multiple independent primary tumors and not metastases from a single primary cell. Thus each tumor derives from a different clone of cells and therefore likely represents a separate infection event.…”

C2 Primary leiomyoma in an immunocompetent woman: A case report and review of literature

“…Recently, the causality of Epstein-Barr virus is implicated in the etiology of the leiomyoma, especially in immunodeficiency states. [6] Some of these tumors are seen to occur in uncommon locations, further strengthening the association of immunodeficiency and the neoplasm. [3]…”

Oesophageal mesenchymal tumours: clinicopathological features and absence of Epstein-Barr virus

“…The smooth muscle tumours developed in these patients were often located in the liver and lung and sometimes in spleen and adrenal gland. [1][2][3][4][5][6][7][8][9][10][11][12] In gastrointestinal tract, these EBV related smooth muscle tumours have been reported in stomach and in large and small intestine. To our knowledge, no such case has been reported in the oesophagus.…”