Bonatti H, Hoefer D, Rogatsch H, Margreiter R, Larcher C, Antretter H
Department of General and Transplant Surgery, University Hospital, Innsbruck, Austria.
Transplant Proc. 2005 May;37(4):1839-44. doi: 10.1016/j.transproceed.2005.03.142.
In contrast to Epstein-Barr virus (EBV)-associated posttransplant lymphoproliferative disorders (PTLD), EBV-associated leiomyomatous tumors have thus far only rarely been described.
Two years after heart transplantation with ATG induction, cyclosporine (CsA; trough levels of 250 ng/mL)-based triple drug immunosuppression), a 23-year-old patient developed a small round lesion within the left lateral liver segment. The patient underwent ultrasound-guided biopsy followed by liver resection. Histological and immunohistological examination showed a leiomyosarcoma. In situ hybridization using EBV-specific EB endoplasmic reticulum-RNA showed an intensive signal in almost all tumor cells. The tumor stained for EB nuclear antigen (EBNA)-2-protein. Immunosuppression was drastically reduced, namely, CsA levels <100 ng/dL, prednisolone 5 mg, azathioprine withdrawn, and antiviral chemotherapy initiated with 10 days of IV gancyclovir and acyclovir followed by oral famcyclovir. During the follow-up, anti-EBV-IgM, anti-early antigen antibodies, and anti-EBNA antibodies were continuously monitored excluding significant EBV replication. Eighteen months post-liver resection, and high-resolution computed tomography scan demonstrated two paravertebral tumors. These lesions and a small nodule at the left ankle were resected revealing identical leiomyosarcomata. Immunosuppression was further reduced (CsA levels 75 ng/dL) and famcyclovir maintenance therapy started. Nevertheless, 2 years later the patient again developed tumor recurrence (perirectal, liver, and right adrenal gland); the tumors were surgically removed. The therapy was switched to Rapamycin and famcyclovir was continued. Three years after the last surgical intervention, the patient is well and recurrence-free.
Long-term survival in patients with posttransplant EBV-associated leiomyosarcoma can be achieved by combined surgical intervention, reduction of immunosuppression, switch to Sirolimus, and antiviral chemotherapy.
与爱泼斯坦-巴尔病毒（EBV）相关的移植后淋巴细胞增生性疾病（PTLD）不同，EBV相关的平滑肌瘤性肿瘤迄今为止仅有极少的病例报道。
一名23岁患者在接受心脏移植并使用抗胸腺细胞球蛋白诱导、基于环孢素（CsA；谷浓度为250 ng/mL）的三联药物免疫抑制治疗两年后，在左肝外侧段出现一个小圆形病变。患者接受了超声引导下活检，随后进行了肝切除术。组织学和免疫组织学检查显示为平滑肌肉瘤。使用EBV特异性EB内质网-RNA的原位杂交显示几乎所有肿瘤细胞中都有强烈信号。肿瘤细胞EB核抗原（EBNA）-2蛋白染色呈阳性。免疫抑制大幅降低，即CsA水平<100 ng/dL，泼尼松龙5 mg，停用硫唑嘌呤，并开始抗病毒化疗，静脉注射更昔洛韦和阿昔洛韦10天，随后口服泛昔洛韦。在随访期间，持续监测抗EBV-IgM、抗早期抗原抗体和抗EBNA抗体，排除明显的EBV复制。肝切除术后18个月，高分辨率计算机断层扫描显示两个椎旁肿瘤。切除了这些病变以及左脚踝处的一个小结节，结果显示为相同的平滑肌肉瘤。免疫抑制进一步降低（CsA水平75 ng/dL）并开始泛昔洛韦维持治疗。然而，2年后患者再次出现肿瘤复发（直肠周围、肝脏和右肾上腺）；手术切除了肿瘤。治疗改为雷帕霉素，并继续使用泛昔洛韦。最后一次手术干预三年后，患者情况良好，无复发。
移植后EBV相关平滑肌肉瘤患者通过联合手术干预、降低免疫抑制、改用西罗莫司和抗病毒化疗可实现长期生存。
