系统性红斑狼疮患者的肝脏血管周围上皮样细胞瘤
Liver perivascular epithelioid cell tumor in a patient with systemic lupus erythematosus.
作者信息
Voulgari Paraskevi V, Tatsi Vissaria, Milionis Haralampos J, Goussia Anna, Xydis Vasileios, Glantzounis George K
机构信息
Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Ioannina, Greece.
Department of Surgery, Medical School, University of Ioannina, Ioannina, Greece.
出版信息
Int J Surg Case Rep. 2018;53:193-195. doi: 10.1016/j.ijscr.2018.10.063. Epub 2018 Nov 1.
INTRODUCTION
Perivascular epithelioid cell tumor (PECOMA) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers showing a benign course,although malignant tumors have also been reported. To date there are approximately 33 cases of published hepatic pecomas.
PRESENTATION OF CASE
We describe a 47-year-old man with a 27-year past medical history of systemic lupus erythematosus (SLE) who underwent left liver lobectomy due to a liver pecoma. His postoperative course complicated with infection, thrombosis of hepatic artery and liver ischemia as well as drug fever.
DISCUSSION
Treatment protocol especially for hepatic PECOMA has not reached a consensus although surgical resection is the preferred therapy.
CONCLUSION
This is the first case of coexistence of liver pecoma and SLE.
引言
血管周上皮样细胞肿瘤（PECOMA）是一种罕见的间叶性肿瘤，表达肌源性和黑素细胞标志物，通常呈良性病程，不过也有恶性肿瘤的报道。迄今为止，已发表的肝PECOMA病例约有33例。
病例介绍
我们描述了一名47岁男性，有27年系统性红斑狼疮（SLE）病史，因肝PECOMA接受了左肝叶切除术。他的术后病程出现了感染、肝动脉血栓形成、肝脏缺血以及药物热等并发症。
讨论
尽管手术切除是首选治疗方法，但对于肝PECOMA的治疗方案尚未达成共识。
结论
这是首例肝PECOMA与SLE并存的病例。
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