Dekate Jyoti, Chetty Runjan
From the Department of Pathology, Laboratory Medicine Program, University Health Network and University of Toronto, Toronto, Ontario, Canada.
Arch Pathol Lab Med. 2016 Jul;140(7):718-22. doi: 10.5858/arpa.2015-0120-RS.
Immunodeficient individuals are prone to develop a number of opportunistic infections and unique neoplasms. Epstein-Barr virus-associated smooth muscle tumor is an uncommon neoplasm associated with immunodeficiency. It has been described in patients infected with human immunodeficiency virus, in the posttransplant setting, and in those with congenital immunodeficiency. Different anatomic sites can be involved by Epstein-Barr virus-associated smooth muscle tumor, and even multiple locations can contain these unique lesions within the same patient. The presence of variable numbers of intratumoral lymphocytes and primitive round cell areas are the unique defining features for this tumor. Histopathologic features may vary considerably in terms of cellular atypia, mitotic activity, and necrosis, with no correlation to the clinical behavior. Demonstration of Epstein-Barr virus infection by in situ hybridization within tumor cell remains critical for the diagnosis. The mechanism for Epstein-Barr virus infection of progenitor cells and neoplastic transformation has been an area of interest and conjecture. Different treatment strategies are proposed according to underlying disease status. This paper reviews the clinicopathologic features of this uncommon neoplasm with detailed discussion of the role of Epstein-Barr virus in the pathogenesis.
免疫缺陷个体容易发生多种机会性感染和独特的肿瘤。爱泼斯坦-巴尔病毒相关的平滑肌瘤是一种与免疫缺陷相关的罕见肿瘤。它已在感染人类免疫缺陷病毒的患者、移植后环境以及先天性免疫缺陷患者中被描述。爱泼斯坦-巴尔病毒相关的平滑肌瘤可累及不同的解剖部位，甚至同一患者的多个部位可出现这些独特病变。肿瘤内淋巴细胞数量可变以及原始圆形细胞区域的存在是该肿瘤独特的定义特征。组织病理学特征在细胞异型性、有丝分裂活性和坏死方面可能有很大差异，与临床行为无关。通过原位杂交在肿瘤细胞内证实爱泼斯坦-巴尔病毒感染对诊断仍然至关重要。爱泼斯坦-巴尔病毒感染祖细胞和肿瘤转化的机制一直是一个备受关注和猜测的领域。根据潜在疾病状态提出了不同的治疗策略。本文回顾了这种罕见肿瘤的临床病理特征，并详细讨论了爱泼斯坦-巴尔病毒在发病机制中的作用。
