原发性肝血管周围上皮样细胞瘤(PEComa)是一种极为罕见的实体。在这篇文章中,我们提供了1例病例报告,并对诊断为原发性肝脏PEComa的患者的文献进行了综述,并详细阐述了诊断和治疗方式.使用术语“血管周围上皮样细胞肿瘤”进行了系统的文献检索,\"PEComa\",\"肝脏\",和“肝”。包括所有描述诊断为原发性肝PEComa的患者的文章。我们从75篇文章中确定了224例原发性肝PEComa患者,并从本研究中确定了1例,其中女性占优势(比例为4:1),平均年龄为45.3±12.1岁。大多数患者(224人中有114人,50.9%)无症状。共有183例(81.3%)患者在确诊时接受了外科肝切除术,19人(8.4%)接受了监测。在7例(3.1%)和6例(2.7%)患者中检测到复发和转移,分别。总之,手术切除仍然是治疗的基石;然而,非特异性影像学特征的存在使得术前难以明确诊断.因此,多学科方法应该是选择治疗方式的黄金标准。
A primary liver perivascular epithelioid cell tumor (PEComa) is an extremely rare entity. In this article, we present a case report with a
review of the literature on the patients diagnosed with primary liver PEComa and an elaboration of diagnostic and treatment modalities. A systematic literature search was conducted using the terms \"perivascular epithelioid cell tumor\", \"PEComa\", \"liver\", and \"hepatic\". All articles describing patients diagnosed with primary liver PEComa were included. We identified a total of 224 patients of primary liver PEComa from 75 articles and a case from the present study with a significant preponderance of females (ratio 4:1) and with a mean age of 45.3 ± 12.1 years. Most of the patients (114 out of 224, 50.9%) were asymptomatic. A total of 183 (81.3%) patients underwent surgical hepatic resection at the time of diagnosis, while 19 (8.4%) underwent surveillance. Recurrence and metastases were detected in seven (3.1%) and six (2.7%) patients, respectively. In conclusion, surgical resection remains the cornerstone of therapy; however, the presence of nonspecific imaging features makes it difficult to reach a definite diagnosis preoperatively. Therefore, a multidisciplinary approach should be the gold standard in selecting the treatment modality.