Khan Afshin A, Estfan Bassam N, Yalamanchali Anirudh, Niang Djibril, Savage Erica C, Fulmer Clifton G, Gosnell Hailey L, Modaresi Esfeh Jamak
Department of Internal Medicine, Cleveland Clinic Foundation, Cleveland, OH 44195, United States.
Department of Solid Tumor Oncology, Taussig Cancer Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, United States.
World J Clin Oncol. 2022 Jun 24;13(6):540-552. doi: 10.5306/wjco.v13.i6.540.
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.
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.
It is imperative to continue building on this knowledge and keeping EBV-SMTs on the differential in immunocompromised individuals.
爱泼斯坦-巴尔病毒相关平滑肌肿瘤(EBV-SMT)是一种罕见的肿瘤实体。然而,随着器官移植和免疫抑制频率的增加,EBV-SMT的发病率正在上升。EBV-SMT的诊断依赖于组织病理学和免疫化学染色,以将其与移植后淋巴细胞增生性疾病(PTLD)区分开来。对于EBV-SMT的治疗尚无明确共识。然而,手术切除、化疗、放疗和降低免疫抑制等方法已被探索,且取得了不同程度的成功。
我们的病例系列包括6例不同年龄组的EBV-SMT病例,采用了不同的治疗方式,这为关于这种罕见肿瘤的有限现有文献增添了内容。免疫抑制与疾病诊断之间的中位潜伏时间为4年。EBV-SMT表现出不同程度的侵袭性,在肿瘤多发和免疫功能较差的患者中似乎临床结局更差。
必须继续基于这一知识体系,并在免疫功能低下个体的鉴别诊断中考虑EBV-SMT。