We present a patient (male 54 years) with a history of renal transplant who 14 years post
transplantation developed a symptomatic (
stridor) laryngeal Epstein Barr virus (EBV)-associated
smooth muscle tumor (EBV-SMT) in the absence of concomitant disease elsewhere. Nine years post
transplantation the patient developed a subcutaneous EBV-SMT
tumor located on the calf. The laryngeal
tumor displayed low-grade nuclear atypia and was infiltrating into the surrounding soft tissue, focally ulcerating through the overlying epithelium. Histologic features included: neoplastic cells with myoid differentiation, a component of primitive appearing small mesenchymal cells with hyperchromatic nuclei, mitotic activity, intralesional small to medium sized blood vessels and T-lymphocytes. Both the myoid and small cell mesenchymal components strongly expressed smooth muscle actin and h-
caldesmon, but not
desmin, cytokeratins, CD34 or
S-100 protein. There was strong positive nuclear reaction for EBV-
RNA on in situ hybridization (EBER). No other
tumor was detected on clinical and radiological examinations and no evidence of
tumor in other sites, over 8 months of follow-up, till death was detected. This case emphasizes the importance of considering this pathologic entity when solitary smooth muscle actin-expressing
tumors are encountered in the larynx of immunocompromised patients.