Department of Oral and Maxillofacial Clinical Science, Faculty of Dentistry, Universiti Malaya, Kuala Lumpur, Malaysia.
Department of Oral Pathology and Oral Medicine, Hospital Queen Elizabeth, Kota Kinabalu, Sabah, Malaysia.
Head Neck Pathol. 2023 Sep;17(3):821-825. doi: 10.1007/s12105-023-01560-y. Epub 2023 May 20.
EBV-associated smooth muscle tumors (EBV-SMTs) are rare and typically develop in individuals with a compromised immune system, particularly those who have acquired immunodeficiency syndrome (AIDS) or who have undergone organ transplants.
We document a case of EBV-SMT in an HIV-positive 25-year-old man. The lesion was incised and assessed histologically and a panel of immune markers were performed. EBV association was demonstrated by in situ hybridization for EBV-encoded RNA (EBER-ISH).
Microscopically, the tumor composed of mildly pleomorphic, ovoid to spindled cells with numerous slit-like vascular channels. The tumor cells exhibited diffuse and strong immunoreactivity for smooth muscle actin (SMA) and focal positivity for h-caldesmon. EBER-ISH of the tumor cells revealed strong positive nuclear signals.
The histopathological features of EBV-SMT do not conform to either benign or malignant SMTs and it has a peculiar predilection to develop at sites unusual for leiomyoma or leiomyosarcoma. The key diagnostic features of EBV-SMT include history of immunosuppression, histologic evidence of primitive and mildly pleomorphic cells maintaining blunt nuclear features in most areas, and positivity for EBER-ISH.
EB 病毒相关平滑肌肿瘤（EBV-SMT）罕见，通常发生在免疫系统受损的个体中，特别是那些患有获得性免疫缺陷综合征（AIDS）或接受过器官移植的个体。
我们记录了一例 HIV 阳性 25 岁男性的 EBV-SMT 病例。对病变进行了切开评估，并进行了一系列免疫标志物检测。通过原位杂交检测 EBV 编码的 RNA（EBER-ISH）来证明 EBV 相关性。
显微镜下，肿瘤由轻度多形性、卵圆形至梭形细胞组成，伴有许多裂隙状血管通道。肿瘤细胞弥漫且强烈表达平滑肌肌动蛋白（SMA），并灶性表达 h-钙调蛋白。肿瘤细胞的 EBER-ISH 显示出强烈的阳性核信号。
EBV-SMT 的组织病理学特征不符合良性或恶性 SMT，它具有在平滑肌瘤或平滑肌肉瘤不常见部位发生的特殊倾向。EBV-SMT 的关键诊断特征包括免疫抑制史、在大多数区域保持钝核特征的原始和轻度多形性细胞的组织学证据，以及 EBER-ISH 阳性。
