Copyright
©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
Epstein-Barr virus-associated smooth muscle tumors in immunocompromised patients: Six case reports
Afshin A Khan, Bassam N Estfan, Anirudh Yalamanchali, Djibril Niang, Erica C Savage, Clifton G Fulmer, Hailey L Gosnell, Jamak Modaresi Esfeh
Afshin A Khan, Anirudh Yalamanchali, Djibril Niang, Department of Internal Medicine, Cleveland Clinic Foundation, Cleveland, OH 44195, United States
Bassam N Estfan, Department of Solid Tumor Oncology, Taussig Cancer Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, United States
Erica C Savage, Department of Pathology, Cleveland Clinic, Cleveland, OH 44195, United States
Clifton G Fulmer, Hailey L Gosnell, Department of Pathology, Cleveland Clinic Foundation, Cleveland, OH 44195, United States
Jamak Modaresi Esfeh, Department of Gastroenterology, Hepatology and Nutrition , Cleveland Clinic, Cleveland, OH 44195, United States
Author contributions: Khan AA wrote the manuscript; Bassam BN and Esfeh JM conceptualized and proofread the paper; Yalamanchali A and Niang D contributed to the paper; Savage E, Fulmer CG, and Gosnell HL provided input on pathology including preparing slides; and All authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from all six patients or healthcare power of attorney, where applicable, for the publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016). The manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
https://creativecommons.org/Licenses/by-nc/4.0/ Corresponding author: Afshin A Khan, MD, Doctor, Department of Internal Medicine, Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH 44195, United States. khana13@ccf.org
Received: January 12, 2022
Peer-review started: January 12, 2022
First decision: March 24, 2022
Revised: April 6, 2022
Accepted: May 12, 2022
Article in press: May 12, 2022
Published online: June 24, 2022
BACKGROUND
Epstein-Barr virus associated smooth muscle tumor (EBV-SMT) is a rare oncological entity. However, there is an increasing incidence of EBV-SMTs, as the frequency of organ transplantation and immunosuppression grows. EBV-SMT diagnosis relies on histopathology and immunochemical staining to distinguish it from post-transplant lymphoproliferative disorder (PTLD). There is no clear consensus on the treatment of EBV-SMTs. However, surgical resection, chemotherapy, radiation therapy, and immunosuppression reduction have been explored with varying degrees of success.
CASE SUMMARY
Our case series includes six cases of EBV-SMTs across different age groups, with different treatment modalities, adding to the limited existing literature on this rare tumor. The median latency time between immunosuppression and disease diagnosis is four years. EBV-SMTs present with variable degrees of aggressiveness and seem to have worse clinical outcomes in patients with tumor multiplicity and worse immunocompetency.
CONCLUSION
It is imperative to continue building on this knowledge and keeping EBV-SMTs on the differential in immunocompromised individuals.
Keywords: Epstein-Barr virus, Smooth muscle tumors, Human immunodeficiency virus, Epstein-Barr virus-associated smooth muscle tumors, Immunocompromised, Solid Organ Transplant, Orthotopic heart transplant, Orthotopic liver transplant, Living related kidney transplant, Post-transplant lymphoproliferative disorders, Case report
Core Tip: Epstein-Barr virus associated smooth muscle tumor (EBV-SMT) is a rare oncological entity. Only a handful of case series have shed light on the presence of EBV-SMT in individuals, most of whom are immunocompromised. EBV-SMT should not be confused with post-transplant lymphoproliferative disorder. Histopathology should help guide the diagnosis.
