EBV-Associated Smooth Muscle Tumor in the Malar Area of an HIV-Positive Patient: A Case Report

Head Neck Pathol. 2023 Sep;17(3):821-825. doi: 10.1007/s12105-023-01560-y. Epub 2023 May 20.

Abstract

Background: EBV-associated smooth muscle tumors (EBV-SMTs) are rare and typically develop in individuals with a compromised immune system, particularly those who have acquired immunodeficiency syndrome (AIDS) or who have undergone organ transplants.

Methods: We document a case of EBV-SMT in an HIV-positive 25-year-old man. The lesion was incised and assessed histologically and a panel of immune markers were performed. EBV association was demonstrated by in situ hybridization for EBV-encoded RNA (EBER-ISH).

Results: Microscopically, the tumor composed of mildly pleomorphic, ovoid to spindled cells with numerous slit-like vascular channels. The tumor cells exhibited diffuse and strong immunoreactivity for smooth muscle actin (SMA) and focal positivity for h-caldesmon. EBER-ISH of the tumor cells revealed strong positive nuclear signals.

Conclusion: The histopathological features of EBV-SMT do not conform to either benign or malignant SMTs and it has a peculiar predilection to develop at sites unusual for leiomyoma or leiomyosarcoma. The key diagnostic features of EBV-SMT include history of immunosuppression, histologic evidence of primitive and mildly pleomorphic cells maintaining blunt nuclear features in most areas, and positivity for EBER-ISH.

Keywords: EBV-SMT; HIV positive; Head and neck; Immunocompromised; Smooth muscle neoplasm.

Publication types

  • Case Reports

MeSH terms

  • Acquired Immunodeficiency Syndrome*
  • Adult
  • Epstein-Barr Virus Infections* / complications
  • Epstein-Barr Virus Infections* / pathology
  • Herpesvirus 4, Human
  • Humans
  • Leiomyosarcoma* / pathology
  • Male
  • Smooth Muscle Tumor* / pathology