Kazmi Syed A Jaffar, Aizenberg Michele R, Harper James L, McComb Rodney D
1University of Nebraska Medical Center, Omaha, NE, USA.
Int J Surg Pathol. 2014 Apr;22(2):186-9. doi: 10.1177/1066896913494793. Epub 2013 Jul 9.
Epstein-Barr virus-associated smooth muscle tumors (EBV-SMTs) are rare lesions that occur in immunocompromised patients. Dural involvement appears to be less common in organ transplant recipients than in HIV patients. Due to the paucity of reported cases following organ transplantation, the natural history of these lesions is unclear. We describe an 8-year-old female who presented with adrenal, small bowel, and intracranial tumors 6 years following renal transplantation. Histopathological analysis revealed a highly cellular, mitotically active, smooth muscle neoplasm without necrosis. The tumor stained diffusely for smooth muscle actin and myosin. In situ hybridization for EBV-encoded RNA was diffusely positive. Following gross total resection, antiviral therapy, and a reduction in immunosuppression, the patient is tumor-free at 3 years follow-up. In patients with compromised immune systems, it is important to recognize this unique form of SMT because, even when there are multiple lesions, the prognosis may be excellent.
爱泼斯坦-巴尔病毒相关的平滑肌肿瘤（EBV-SMTs）是发生于免疫功能低下患者的罕见病变。硬脑膜受累在器官移植受者中似乎比在HIV患者中少见。由于器官移植后报告的病例较少，这些病变的自然病程尚不清楚。我们描述了一名8岁女性，在肾移植6年后出现肾上腺、小肠和颅内肿瘤。组织病理学分析显示为高度细胞性、有丝分裂活跃的平滑肌肿瘤，无坏死。肿瘤平滑肌肌动蛋白和肌球蛋白弥漫性染色阳性。EBV编码RNA的原位杂交呈弥漫性阳性。在进行肿瘤全切、抗病毒治疗并减少免疫抑制后，患者在3年随访时无肿瘤。对于免疫系统受损的患者，认识到这种独特形式的SMT很重要，因为即使存在多个病变，预后也可能良好。
