Morgello S, Kotsianti A, Gumprecht J P, Moore F
Department of Pathology, The Mount Sinai Medical Center, New York, New York 10029, USA.
J Neurosurg. 1997 May;86(5):883-7. doi: 10.3171/jns.1997.86.5.0883.
A 35-year-old man infected with human immunodeficiency virus presented with cervical myelopathy of 2 months duration. Clinical and radiographic evaluation revealed a discrete, subdural mass at C-6. At surgery, the mass proved to have a dural attachment and thus clinically, radiographically, and grossly, it resembled meningioma. Histopathological analysis revealed a leiomyosarcoma that stained diffusely for muscle-specific actin. Electron microscopy revealed basal lamina surrounding the tumor cells and intracytoplasmic bundles of myofilaments. Epstein-Barr virus (EBV) was demonstrated within tumor cell nuclei by in situ hybridization for EBER1 messenger RNA and immunohistochemical staining for EBNA2 protein. Epstein-Barr virus latent membrane protein (LMP1) was not detected. This is the first documentation of an EBV-associated smooth-muscle tumor of the dura, and the first demonstration that tumors in this location contain EBV in an unusual form of latency not seen in lymphoid cell lines. With increasing numbers of individuals being afflicted with long-term immunosuppression, EBV-associated dural leiomyoma and leiomyosarcoma may be encountered more frequently in the future.
一名35岁的人类免疫缺陷病毒感染者出现了持续2个月的颈髓病。临床和影像学评估显示，在C-6水平有一个孤立的硬膜下肿块。手术中发现该肿块与硬脑膜相连，因此在临床、影像学和大体表现上，它类似于脑膜瘤。组织病理学分析显示为平滑肌肉瘤，肌特异性肌动蛋白呈弥漫性染色。电子显微镜检查显示肿瘤细胞周围有基膜，胞质内有肌丝束。通过EBER1信使核糖核酸原位杂交和EBNA2蛋白免疫组化染色，在肿瘤细胞核内检测到爱泼斯坦-巴尔病毒（EBV）。未检测到爱泼斯坦-巴尔病毒潜伏膜蛋白（LMP1）。这是关于EBV相关的硬脑膜平滑肌肿瘤的首次记录，也是首次证明该部位的肿瘤以一种在淋巴样细胞系中未见的异常潜伏形式含有EBV。随着越来越多的人受到长期免疫抑制的影响，未来可能会更频繁地遇到EBV相关的硬脑膜平滑肌瘤和平滑肌肉瘤。
