Restricted accessCase reportFirst published online 2024-9
EBV-Associated Smooth Muscle Tumors With Autoimmune Hemolytic Anemia and Hepatitis B Infection: Report of a Previously Undescribed Neoplasm With Review
Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is rare in adults. The presence of intratumoral T lymphocytes and primitive rounded cells characterized this neoplasm. We report a 24-year-old Chinese man who developed EBV-SMT in the right adrenal gland with hepatitis B infection and autoimmune hemolytic anemia without a history of HIV infection, primary immune deficiency, organ transplantation, or malignant tumor. This patient had an unknown immunodeficient state. EBV-SMTs are commonly located in the liver, lung, and gastrointestinal tract but rarely in the adrenal gland. We reviewed 10 reported literature on EBV-SMT in the adrenal gland. It is imperative to distinguish EBV-SMT from conventional somatic smooth muscle tumors. The discovery of EBV-SMT forces the clinician to conduct a thorough evaluation of immune function and immune status surveillance, and these patients are vulnerable to subsequent malignant tumors.
LeeESDickmanPJaffeR, et al.Post-transplant spindle cell tumor (PTST): an entity associated with Epstein Barr virus [abstract]. Mod Pathol. 1993;68(1):A127–A127.
2.
LeeESLockerJNalesnikM, et al.The association of Epstein-Barr virus with smooth-muscle tumors occurring after organ transplantation. N Engl J Med. 1995;5(332):19–25. doi:https://doi.org/10.1056/NEJM199501053320104
3.
PetrilliGLorenziLParacchiniR, et al.Epstein-Barr virus-associated adrenal smooth muscle tumors and disseminated diffuse large B-cell lymphoma in a child with common variable immunodeficiency: a case report and review of the literature. Int J Surg Pathol. 2014;22(8):712–721.
4.
GallienSZuberBPolivkaM, et al.Multifocal Epstein-Barr virus-associated smooth muscle tumor in adults with AIDS: case report and review of the literature. Oncology. 2008;74(3–4):167–176.
5.
Jimenez-HeffernanJAHardissonDPalaciosJ, et al.Adrenal gland leiomyoma in a child with acquired immunodeficiency syndrome. Pediatr Pathol Lab Med. 1995;15(6):923–929.
6.
JossenJChuJHotchkissH, et al.Epstein-Barr virus-associated smooth muscle tumors in children following solid organ transplantation: a review. Pediatr Transplant. 2015;199(2):235–243.
7.
Debiec-RychterMCroesRDe VosR, et al.Complex genomic rearrangement of ALK loci associated with integrated human Epstein-Barr virus in a post-transplant myogenic liver tumor. Am J Pathol. 2003;163(3):913–922.
8.
SuankratayCShuangshotiSMutiranguraA, et al.Epstein-Barr virus infection-associated smooth-muscle tumors in patients with AIDS. Clin Infect Dis. 2005;40(10):1521–1528.
9.
DeyrupATLeeVKHillCE, et al.Epstein-Barr virus-associated smooth muscle tumors are distinctive mesenchymal tumors reflecting multiple infection events: a clinicopathologic and molecular analysis of 29 tumors from 19 patients. Am J Surg Pathol. 2006;30(1):75–82.
10.
SathePAShahHUKothariKS, et al.Bilateral Epstein-Barr virus-associated adrenal leiomyomas in a child without an established immunodeficiency. Pediatr Dev Pathol. 2012;15(4):329–332.
11.
AdamEWangLHerringtonC, et al.Synchronous HIV/AIDS-related Epstein-Barr virus-associated smooth muscle tumors in a 20-year-old female. Pediatr Infect Dis J. 2014;33(10):1055–1056.
12.
BrownDADeepNLDriscollCL, et al.Synchronous Epstein-Barr virus-associated skull base and adrenal smooth-muscle tumors in an 8-year-old girl with recent Epstein-Barr virus infection. J Neurosurg Pediatr. 2018;22(3):283–287.
13.
Arbelo-RamosNAndersonKJoshiA, et al.Heck's disease occurring after Epstein-Barr virus-associated smooth muscle tumors in an immunosuppressed child. Pediatr Dermatol. 2021;38(1):257–259.
14.
ChongYBLuPLMaYC, et al.Epstein-Barr virus-associated smooth muscle tumor and its correlation with CD4 levels in a patient with HIV infection. Front Cell Infect Microbiol. 2022;24(12):725342. doi:https://doi.org/10.3389/fcimb.2022.725342
15.
ShawRKIssekutzACFraserR, et al.Bilateral adrenal EBV-associated smooth muscle tumors in a child with a natural killer cell deficiency. Blood. 2012;119(17):4009–4012.
16.
WatanabeRSchafernakKTSoaresFA. Soft Tissue Tumours. WHO Classification of Tumours·5th Edition, Soft Tissue and Bone Tumours, Lyon: International Agency for Research on Cancer, 2020, 190–192.
17.
TanCSLohHLFooMW, et al.Epstein-Barr virus-associated smooth muscle tumors after kidney transplantation: treatment and outcomes in a single center. Clin Transplant. 2013;27(4):E462–E468. doi:https://doi.org/10.1111/ctr.12139
MantadakisEChatzimichaelEKontekakiE, et al.EBV-related cold agglutinin disease presenting with conjugated hyperbilirubinemia: a pediatric case report and mini review. J Pediatr Hematol Oncol. 2019;41(4):324–327.
20.
GroudanKevinNuelle, et al.Warm autoimmune hemolytic anemia secondary to acute hepatitis B virus infection. Am J Gastroenterol. 2021;116:S1163–S1164.
