Department of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan.
Division of Pathophysiology, Okayama University Graduate School of Health Sciences, Okayama 700-8558, Japan.
Int J Mol Sci. 2021 Jan 21;22(3):1053. doi: 10.3390/ijms22031053.
Epstein-Barr virus (EBV)-positive mucocutaneous ulcer (EBVMCU) was first described as a lymphoproliferative disorder in 2010. EBVMCU is a unifocal mucosal or cutaneous ulcer that often occurs after local trauma in patients with immunosuppression; the patients generally have a good prognosis. It is histologically characterized by proliferating EBV-positive atypical B cells accompanied by ulcers. On the basis of conventional pathologic criteria, EBVMCU may be misdiagnosed as EBV-positive diffuse large B-cell lymphoma or other lymphomas. However, its prognosis differs from that of EBV-associated lymphomas, in that patients with EBVMCU frequently show spontaneous regression or complete remission without chemotherapy. Therefore, EBVMCU is now recognized as a low-grade malignancy or a pseudo-malignant lesion. Avoiding unnecessary chemotherapy by distinguishing EBVMCU from other EBV-associated lymphomas will reduce the burden and unnecessary harm on patients. On the basis of these facts, EBVMCU was first described as a new clinicopathological entity by the World Health Organization in 2017. In this review, we discuss the clinicopathological characteristics of previously reported EBVMCU cases, while focusing on up-to-date clinical, pathological, and genetic aspects.
2010 年，EB 病毒阳性黏膜溃疡（EBVMCU）首次被描述为一种淋巴增生性疾病。EBVMCU 是一种局灶性黏膜或皮肤溃疡，常发生于免疫抑制患者局部创伤后；患者预后一般良好。其组织学特征为增生的 EBV 阳性非典型 B 细胞伴溃疡形成。在常规病理标准基础上，EBVMCU 可能误诊为 EBV 阳性弥漫性大 B 细胞淋巴瘤或其他淋巴瘤。然而，其预后与 EBV 相关淋巴瘤不同，EBVMCU 患者常自发性消退或完全缓解而无需化疗。因此，EBVMCU 现被认为是一种低度恶性肿瘤或假性恶性病变。通过将 EBVMCU 与其他 EBV 相关淋巴瘤相鉴别，避免不必要的化疗，可减轻患者负担和不必要的伤害。基于这些事实，世界卫生组织于 2017 年首次将 EBVMCU 描述为一种新的临床病理实体。在本综述中，我们讨论了既往报道的 EBVMCU 病例的临床病理特征，同时重点关注最新的临床、病理和遗传学方面。
