Abstract
Epstein–Barr virus positive (EBV+) smooth muscle tumors (SMTs) constitute a very rare oncological entity. They usually develop in the context of secondary immunodeficiency caused by human immunodeficiency virus infection or immunosuppressive treatment after solid organ transplantation. However, in a small fraction of predominantly pediatric patients, EBV+ SMTs may occur in patients with primary immunodeficiency disorders (PIDs), such as GATA2 and CARMIL2 deficiency. In secondary immunodeficiencies and when the underlying condition can not be cured, the treatment of EBV+ SMTs is based on surgery in combination with antiretroviral and reduced or altered immunosuppressive pharmacotherapy, respectively. Importantly, without definitive reconstitution of cellular immunity, long-term survival is poor. This is particularly relevant for patients with EBV+ SMTs on the basis of PIDs. Recently, allogeneic hematopoietic stem cell transplantation resulted in cure of immunodeficiency and EBV+ SMTs in a GATA2-deficient patient. We propose that in the absence of secondary immunodeficiency disorders patients presenting with EBV+ SMTs should be thoroughly evaluated for PIDs. Allogeneic hematopoietic stem cell transplantation should be taken into consideration, ideally in the setting of a prospective clinical trial.
Highlights
Epstein–Barr virus (EBV) is a gamma 1 herpes virus that preferentially infects human epithelial cells of the oropharynx and B cells of the adaptive immune system to establish lifelong latency [1]
EBV+ SMTs constitute very rare tumors seen in the context of secondary immunodeficiency disorders (SIDs) caused by human immunodeficiency virus infection or immunosuppressive treatment after solid organ transplantation [11]
The pathogenesis of EBV+ SMTs remains largely unknown, but it is evident that an immunocompromised host is a conditio sine qua non and that especially T and NK cell immunity is important to prevent the disease [11, 21, 35]
Summary
Epstein–Barr virus (EBV) is a gamma 1 herpes virus that preferentially infects human epithelial cells of the oropharynx and B cells of the adaptive immune system to establish lifelong latency [1]. Smooth muscle tumors (SMTs) represent a heterogeneous group of disorders with a broad pathological spectrum ranging from very common and benign uterine leiomyoma to malignant leiomyosarcoma The latter is characterized by hypercellularity, nuclear atypia, high mitotic rate, and tumor cell necrosis [10]. Most EBV+ SMTs develop at any age in patients with SIDs due to uncontrolled human immunodeficiency virus infection (HIV EBV+ SMTs) and organ transplantation-associated immunosuppressive treatment (PT EBV+ SMTs) [2, 12]. They rarely present in pediatric patients with proven or suspected PID (PID EBV+ SMTs) [11]. Mierau et al [31] Tulbah et al [36] Reyes et al [20] Monforte-Muñoz et al [32] Hatano et al [19] Atluri et al [30] Vinh et al [37] Shaw et al [35] Petrilli et al [34]
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