Hepatic perivascular epithelioid cell tumor in three patients
Hepatic perivascular epithelioid cell tumor in three patients
复制标题
肝血管周围上皮样细胞瘤三例
DOI：
10.1016/s1499-3872(16)60077-2
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发表时间：
2016-12-01
影响因子：
3.3
通讯作者：
Zhang, Feng
中科院分区：
文献类型：
--
作者：
Hao, Bao-Bin;Rao, Jian-Hua;Zhang, Feng
血管周围上皮样细胞瘤是一种罕见的软组织肿瘤，可发生在不同的位置。本报告包括3例肝PEComa患者（1例男性和2例女性;年龄范围，25-51岁）。收集的资料包括临床表现、诊断、处理、治疗和预后。由于难以通过成像诊断肝PEComa，因此通过肿瘤组织检查（如免疫组织化学）诊断患者，所有载玻片上的HMB-45、Melan-A和SMA均呈阳性。肿瘤由不同的组织组成，包括平滑肌、脂肪组织和厚壁血管。在随访期间，其中一个肿瘤是恶性的（CD 34和Ki-67双阳性），并在手术后3个月复发。此外，在文献中对恶性肝PEComa进行了综述，表明大多数肝PEComa是良性的，但在伴有腹部不适和疼痛、肿瘤尺寸较大（>10 cm）或CD 34和Ki-67阳性染色的老年女性患者（>50岁）中，很少有肝PEComa表现出恶性行为。总之，没有有效的方法来诊断PEComa。目前，PEComa的诊断依赖于免疫组化染色。肿瘤切除和密切随访是治疗PEComa的主要方法。
Perivascular epithelioid cell tumor (PEComa) is a rare, soft tissue tumor that can occur in various locations. The present report included three patients (one male and two females; age range, 25-51 years) with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant (double-positive for CD34 and Ki-67) and recurred 3 months after surgery. In addition, malignant hepatic PEComas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients (>50 years of age) with abdominal discomfort and pain, larger tumor size (>10 cm), or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.