Related Experiment Video
Updated: May 9, 2026
Establishment of Epstein-Barr Virus Growth-transformed Lymphoblastoid Cell Lines
Published on: November 8, 2011
Multifocal histologically malignant Epstein-Barr virus-associated smooth muscle tumor in a pediatric transplant
Syed A Jaffar Kazmi1, Michele R Aizenberg, James L Harper
11University of Nebraska Medical Center, Omaha, NE, USA.
Abstract:
Epstein-Barr virus-associated smooth muscle tumors (EBV-SMTs) are rare lesions that occur in immunocompromised patients. Dural involvement appears to be less common in organ transplant recipients than in HIV patients. Due to the paucity of reported cases following organ transplantation, the natural history of these lesions is unclear. We describe an 8-year-old female who presented with adrenal, small bowel, and intracranial tumors 6 years following renal transplantation. Histopathological analysis revealed a highly cellular, mitotically active, smooth muscle neoplasm without necrosis. The tumor stained diffusely for smooth muscle actin and myosin. In situ hybridization for EBV-encoded RNA was diffusely positive. Following gross total resection, antiviral therapy, and a reduction in immunosuppression, the patient is tumor-free at 3 years follow-up. In patients with compromised immune systems, it is important to recognize this unique form of SMT because, even when there are multiple lesions, the prognosis may be excellent.
Insights
Epstein-Barr virus-associated smooth muscle tumors (EBV-SMTs) are rare in transplant patients. Early recognition and treatment can lead to excellent prognoses for these EBV-SMTs.
Area of Science:
- Oncology
- Virology
- Transplantation Immunology
Background:
- Epstein-Barr virus-associated smooth muscle tumors (EBV-SMTs) are uncommon in immunocompromised individuals.
- Dural involvement is less frequent in organ transplant recipients compared to HIV patients.
- The natural history of EBV-SMTs post-organ transplantation remains poorly understood due to limited case reports.
Observation:
- A case of an 8-year-old female renal transplant recipient is presented.
- The patient developed adrenal, small bowel, and intracranial tumors six years post-transplantation.
- Histopathology confirmed a highly cellular, mitotically active smooth muscle neoplasm with diffuse EBV-encoded RNA positivity.
Findings:
- Tumor cells stained positive for smooth muscle actin and myosin.
- Complete surgical resection, antiviral therapy, and reduced immunosuppression were administered.
- The patient achieved a tumor-free status at a 3-year follow-up.
Implications:
- This case highlights the importance of recognizing EBV-SMTs in immunocompromised patients, including organ transplant recipients.
- Despite multiple lesions, a favorable prognosis is possible with appropriate management.
- Early diagnosis and multimodal treatment strategies are crucial for managing EBV-SMTs in this population.
