Epstein-Barr Virus-Associated Smooth Muscle Tumor of the Spine After Bone Marrow Transplant: Case Report and Review of Literature

World Neurosurg. 2020 Mar:135:192-196. doi: 10.1016/j.wneu.2019.11.160. Epub 2019 Dec 3.

Abstract

Background: Epstein-Barr virus-associated smooth muscle tumors (SMTs) are rare neoplasms that have been found to develop in immunocompromised patients. Three distinct groups of affected patients have been described: (1) human immunodeficiency virus-infected patients, (2) post-transplant patients, and (3) patients with congenital immunodeficiency. The tumors can develop anywhere in the body, with 17 reported cases occurring in the spinal canal, all in patients with human immunodeficiency virus infection.

Case description: We report the first case of Epstein-Barr virus-associated SMT affecting the spinal canal in a post-bone marrow transplant adult patient. Interestingly, unlike other reported cases, the patient described here had not been receiving immunosuppressive therapy in the 2 years prior to diagnosis of the tumor.

Conclusions: Despite the growing number of case reports, this diagnosis presents a challenge, as the pathophysiology and optimal treatment regimens are not well understood. Results of a literature review of Epstein-Barr virus-associated SMT of the spine as well as a discussion of the presentation, management, and prognosis of this condition is presented here.

Keywords: Bone marrow transplant; Epstein-Barr virus; Smooth muscle tumor; Spine.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Decompression, Surgical
  • Epstein-Barr Virus Infections / immunology*
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Immunocompromised Host*
  • Immunosuppressive Agents / adverse effects
  • Lymphoma, Large B-Cell, Diffuse / therapy
  • Male
  • Middle Aged
  • Optic Neuritis / drug therapy
  • Smooth Muscle Tumor / diagnostic imaging*
  • Smooth Muscle Tumor / immunology
  • Smooth Muscle Tumor / surgery
  • Smooth Muscle Tumor / virology
  • Spinal Fusion
  • Spinal Neoplasms / diagnostic imaging*
  • Spinal Neoplasms / immunology
  • Spinal Neoplasms / surgery
  • Spinal Neoplasms / virology
  • Thoracic Vertebrae

Substances

  • Immunosuppressive Agents