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Epstein Barr Virus Associated Smooth Muscle Tumors in Pediatric Heart Transplantation Patients: A Case Series and Review
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  • Jamie Pruitt,
  • Gerard Boyle,
  • Colleen Nasman,
  • Josephine Kam Tai Dermawan,
  • Rabi Hanna,
  • Aron Flagg,
  • Sarah Fracci,
  • John Goldblum,
  • seth rotz
Jamie Pruitt
Cleveland Clinic Foundation
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Gerard Boyle
Cleveland Clinic Foundation
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Colleen Nasman
Cleveland Clinic Foundation
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Josephine Kam Tai Dermawan
Cleveland Clinic Foundation
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Rabi Hanna
Cleveland Clinic
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Aron Flagg
Yale-New Haven Hospital
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Sarah Fracci
Loyola University Chicago
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John Goldblum
Cleveland Clinic
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seth rotz
Cleveland Clinic Foundation

Corresponding Author:rotzs@ccf.org

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Abstract

Background: Epstein-Barr virus associated smooth muscle tumors (EBV-SMT) are rare neoplasms that occur in immunocompromised individuals. Although generally less aggressive in adults, data in pediatric solid organ transplant (SOT) recipients suggest these malignancies may be more difficult to manage. As pediatric SOT becomes more common, the occurrence of EBV-SMT is likely to increase. Understanding what incites and drives these tumors is paramount. Design/Methods: We retrospectively reviewed the electronic health record (EHR) of 152 pediatric cardiac transplant recipients at our single institution between 1985-2017. Three patients were identified as having EBV SMT. We reviewed their demographics, time to malignancy, histology, treatment, and subsequent course. Results: Of the 152 cardiac transplant recipients reviewed at our institution between 1985-2017, three were identified to have EBV SMT (confirmed by EBER CISH). In our series, the average time to diagnosis was approximately 32 months following transplant. Liver was the most common site of EBV SMT. Two patients were diagnosed with EBV SMT after a known PTLD diagnosis. All three patients had elevated EBV titers at (or around) time of diagnosis, and there was one patient with EBV donor/recipient mismatch. Treatment was individualized and varied case by case. Conclusion: EBV-SMTs are rare in pediatric SOT and are difficult to manage in the setting of required immunosuppression. While there is some literature to suggest the use of mTOR inhibition and CTL therapy is helpful in treatment of EBV SMT, data remains limited given the paucity of cases. At current, there remains no standard of care for treatment of pediatric EBV SMT.