Department of neurosurgery, Chiba University Hospital, Chiba, Japan
Department of neurosurgery, Chiba University Hospital, Chiba, Japan.
BMJ Case Rep. 2020 Oct 30;13(10):e236381. doi: 10.1136/bcr-2020-236381.
Epstein-Barr virus-associated smooth muscle tumour (EBV-SMT) is a rare disease occurring in immunosuppressed patients, such as those with AIDS, post-transplantation immunodeficiency and congenital immunodeficiency. Intracranial EBV-SMT after solid organ transplantation has been reported. However, intracranial lesions after bone marrow transplantation are extremely rare. We report the case of a 47-year-old man with a history of acute myeloid leukaemia and bone marrow transplantation. He had symptoms of trigeminal neuralgia, and MRI revealed a left cavernous sinus tumour. He started taking oral gabapentin, but his symptoms did not improve. We performed transnasal endoscopic surgery. Postoperative MRI showed complete removal of the cavernous sinus lesion. Pathological examination showed spindle-shaped cells positive for smooth muscle markers and EBV-encoded small RNA in situ hybridisation. EBV-SMT was pathologically diagnosed. His symptoms improved after surgery. No tumour recurrence was noted on follow-up MRI after 15 months without adjuvant radiation or chemotherapy.
EBV 相关平滑肌肿瘤（EBV-SMT）是一种发生于免疫抑制患者的罕见疾病，如艾滋病患者、移植后免疫缺陷和先天性免疫缺陷患者。已有报道称，实体器官移植后会发生颅内 EBV-SMT，但骨髓移植后的颅内病变极为罕见。我们报告了一例 47 岁男性，患有急性髓系白血病和骨髓移植。他出现三叉神经痛症状，MRI 显示左侧海绵窦肿瘤。他开始服用口服加巴喷丁，但症状没有改善。我们进行了经鼻内镜手术。术后 MRI 显示海绵窦病变完全切除。病理检查显示梭形细胞阳性表达平滑肌标志物，原位杂交显示 EBV 编码的小 RNA。病理诊断为 EBV-SMT。手术后他的症状得到改善。在没有辅助放疗或化疗的情况下，15 个月后随访 MRI 未发现肿瘤复发。
