The deceased was a teenage boy who had a history of cardiac transplantation several years before he died. . The tumor was found in the cavernous sinus at autopsy. Histologically, the tumor is that of an spindle cell neoplastic proliferation with intermediate grade nuclei and is mitotically active. The tumor cells are strongly positive for smooth muscle actin, muscle specific actin, and focally for desmin. This tumor is most likely to be associated with which of the following virus?

A.  Human papilloma virus (HPV)

B.  Human herpes virus type 8 (HHV8)

C.  JC Virus

D.  Cytomegalovirus (CMV)

E.  Epstein-Barr virus (EBV)

Answer and Discussion: The answer is (E). As per the gross photo and histologic description, this tumor is a leiomyosarcoma. Epstein Barr virus has been associated with lymphoproliferative diseases and solid tumors in immunodeficient patients including HIV patients and transplant recipients. As per one study by Bhatia et al., leiomyosarcomas were the most freauently reported sarcoma in patients with HIV/AIDS, and transplantation and EBV was detected in close to 90% of both groups of patients. HPV is related to uterine cervical cancer and squamous cell carcinoma of the upper respiratory tract. JC virus is associated with progressive multifocal leukoencephalopathy which are typically seen in immune compromised patients including HIV/AIDS patients. CMV has been recently demonstrated to be related with glioblastoma.

Further Reading:

• Bhatia K, Shiels MS, Berg A, Engels EA. Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review. Curr Opin Oncol. 2012 Sep;24(5):537-46.

• Lucas KG, Bao L, Bruggeman R, Dunham K, Specht C. The detection of CMV pp65 and IE1 in glioblastoma multiforme. J Neurooncol. 2011 Jun;103(2):231-8